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söndag 25 januari 2026

Sinkkisormiproteiinit, ZZ -tyyppiset

 

ZNF proteiinit jaetaan 30 alaluokkaan.niissä yksi alaluokka on ZZ -tyyppiset  eli ZZZ.
näitä on taulukoitu 18 kappaletta ja nyt uudessa Duodecimissa mainittiin  myös  yksi ZZ-proteiiniperhe, mutta  se ei ole mukana vanhassa taulukossa ja siihenkin kuuluu useampi jäsen.
 Kirjoitan tähän sen luettelon vanhoista ZZ-  sinkkisormiproteiinita, minkä löydän.

(1) CREBBP  ( 16p13.3)  CREB binding protein,  vanha nimi RSTS ja synonyymisiä nimiä RTS, CBP, KAT3A.
  • CREB Binding Lysine Acetyltransferase 2
  • Histone Lysine Acetyltransferase CREBBP 3 4
  • Protein-Lysine Acetyltransferase CREBBP 3 4
  • Protein Lactyltransferas CREBBP
https://www.genecards.org/cgi-bin/carddisp.pl?gene=CREBBP&keywords=CREBBP

  • This gene is ubiquitously expressed and is involved in the transcriptional coactivation of many different transcription factors. First isolated as a nuclear protein that binds to cAMP-response element binding protein (CREB), this gene is now known to play critical roles in embryonic development, growth control, and homeostasis by coupling chromatin remodeling to transcription factor recognition. The protein encoded by this gene has intrinsic histone acetyltransferase activity and also acts as a scaffold to stabilize additional protein interactions with the transcription complex. This protein acetylates both histone and non-histone proteins. This protein shares regions of very high sequence similarity with protein p300 in its bromodomain, cysteine-histidine-rich regions, and histone acetyltransferase(HAT) domain. Mutations in this gene cause Rubinstein-Taybi syndrome (RTS). Chromosomal translocations involving this gene have been associated with acute myeloid leukemia. Alternative splicing results in multiple transcript variants encoding different isoforms. [provided by RefSeq, Feb 2009]CREBBP (CREB Binding Lysine Acetyltransferase ) is a Protein Coding gene. Diseases associated with CREBBP include Rubinstein-Taybi Syndrome 1 and Menke-Hennekam Syndrome 1. Among its related pathways are Nuclear events mediated by NFE2L2 and Regulation of activated PAK-2p34 by proteasome mediated degradation. Gene Ontology (GO) annotations related to this gene include DNA-binding transcription factor activity and transcription factor binding. An important paralog of this gene is EP300.Acetylates histones, giving a specific tag for transcriptional activation (PubMed:21131905, 24616510). Mediates acetylation of histone H3 at 'Lys-18' and 'Lys-27' (H3K18ac and H3K27ac, respectively) (PubMed:21131905). Also acetylates non-histone proteins, like DDX21, FBL, IRF2, MAFG, NCOA3, POLR1E/PAF53 and FOXO1  Binds specifically to phosphorylated CREB and enhances its transcriptional activity toward cAMP-responsive genes. Acts as a coactivator of ALX1. Acts as a circadian transcriptional coactivator which enhances the activity of the circadian transcriptional activators: NPAS2-BMAL1 and CLOCK-BMAL1 heterodimers (PubMed:14645221). Acetylates PCNA; acetylation promotes removal of chromatin-bound PCNA and its degradation during nucleotide excision repair (NER) (PubMed:24939902). Acetylates POLR1E/PAF53, leading to decreased association of RNA polymerase I with the rDNA promoter region and coding region (PubMed:24207024). Acetylates DDX21, thereby inhibiting DDX21 helicase activity (PubMed:28790157). Acetylates FBL, preventing methylation of 'Gln-105' of histone H2A (H2AQ104me) (PubMed:30540930). In addition to protein acetyltransferase, can use different acyl-CoA substrates, such as lactoyl-CoA, and is able to mediate protein lactylation (PubMed:38128537). Catalyzes lactylation of MRE11 in response to DNA damage, thereby promoting DNA double-strand breaks (DSBs) via homologous recombination (HR) (PubMed:38128537). Functions as a transcriptional coactivator for SMAD4 in the TGF-beta signaling pathway (PubMed:25514493). ( CBP_HUMAN,Q92793 )

    Protein Domains for CREBBP Gene

    InterPro:
    Blocks:
    • Bromodomain signature 
      •  
    • TAZ finger 
      •  
    • Zn-finger, ZZ type 
      •  
    • Coactivator CBP, KIX 
      •  
    • Transcriptional coactivation


(2) DMD (Xp21.2-21.1) , Dystrophin, vanha nimi MRX85. Synonyymit:  BMD,

  • This gene spans a genomic range of greater than 2 Mb and encodes a large protein containing an N-terminal actin-binding domain and multiple spectrin repeats. The encoded protein forms a component of the dystrophin-glycoprotein complex (DGC), which bridges the inner cytoskeleton and the extracellular matrix. Deletions, duplications, and point mutations at this gene locus may cause Duchenne muscular dystrophy (DMD), Becker muscular dystrophy (BMD), or cardiomyopathy. Alternative promoter usage and alternative splicing result in numerous distinct transcript variants and protein isoforms for this gene. [provided by RefSeq, Dec 2016]GeneCards Summary for DMD Gene DMD (Dystrophin) is a Protein Coding gene. Diseases associated with DMD include Muscular Dystrophy, Duchenne Type and Muscular Dystrophy, Becker Type. Among its related pathways are Non-integrin membrane-ECM interactions and Striated muscle contraction pathway. Gene Ontology (GO) annotations related to this gene include calcium ion binding and structural constituent of cytoskeleton. An important paralog of this gene is UTRN. UniProtKB/Swiss-Prot Summary for DMD Gene Anchors the extracellular matrix to the cytoskeleton via F-actin. Ligand for dystroglycan. Component of the dystrophin-associated glycoprotein complex which accumulates at the neuromuscular junction (NMJ) and at a variety of synapses in the peripheral and central nervous systems and has a structural function in stabilizing the sarcolemma. Also implicated in signaling events and synaptic transmission. ( DMD_HUMAN,P11532 )

    Protein Domains for DMD Gene

    InterPro:
    Blocks:
    • Actin-binding, actinin-type 
      •  
    • Calponin-like actin-binding 
      •  
    • Spectrin repeat 
      •  
    • WW domain signature 
      •  
    • Zn-finger, ZZ type

(3) DRP2 (Xq22.1), Dystrophin related protein-2                                                             https://www.genecards.org/cgi-bin/carddisp.pl?gene=DRP2&keywords=DRP2

  • Members of the dystrophin family of proteins perform a critical role in the maintenance of membrane-associated complexes at points of intercellular contact in vertebrate cells. The protein encoded by this gene is predicted to resemble certain short C-terminal isoforms of dystrophin and dystrophin-related protein 1 (DRP1 or utrophin). DRP2 is expressed principally in the brain and spinal cord. Two transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Jan 2010]GeneCards Summary for DRP2 Gene DRP2 (Dystrophin Related Protein 2) is a Protein Coding gene. Diseases associated with DRP2 include Spinal Muscular Atrophy, Type I and Mitochondrial Complex Iv Deficiency, Nuclear Type 1. Among its related pathways are Non-integrin membrane-ECM interactions and Nervous system development. Gene Ontology (GO) annotations related to this gene include calcium ion binding. An important paralog of this gene is DMD.UniProtKB/Swiss-Prot Summary for DRP2 Gene

Required for normal myelination and for normal organization of the cytoplasm and the formation of Cajal bands in myelinating Schwann cells. Required for normal PRX location at appositions between the abaxonal surface of the myelin sheath and the Schwann cell plasma membrane. Possibly involved in membrane-cytoskeleton interactions of the central nervous system. ( DRP2_HUMAN,Q13474 )

Protein Domains for DRP2 Gene

InterPro:
Blocks:
  • Spectrin repeat 
    •  
  • WW domain signature 
    •  
  • Zn-finger, ZZ type


(4) DTNA (18q12.1),  Dystrobrevin alfa. Synonyymit: DTN, DTN-1,-2,-3, NRP3.https://www.genecards.org/cgi-bin/carddisp.pl?gene=DTNA&keywords=DTNA

NCBI Gene Summary for DTNA GeneThe protein encoded by this gene belongs to the dystrobrevin subfamily of the dystrophin family. This protein is a component of the dystrophin-associated protein complex (DPC), which consists of dystrophin and several integral and peripheral membrane proteins, including dystroglycans, sarcoglycans, syntrophins and alpha- and beta-dystrobrevin. The DPC localizes to the sarcolemma and its disruption is associated with various forms of muscular dystrophy. Mutations in this gene are associated with left ventricular noncompaction with congenital heart defects. Multiple alternatively spliced transcript variants encoding different isoforms have been identified for this gene. [provided by RefSeq, Jul 2008]

GeneCards Summary for DTNA Gene DTNA (Dystrobrevin Alpha) is a Protein Coding gene. Diseases associated with DTNA include Myopathy With Myalgia, Increased Serum Creatine Kinase, And With Or Without Episodic Rhabdomyolysis 2 and Left Ventricular Noncompaction 1. Among its related pathways are Non-integrin membrane-ECM interactions and Collagen chain trimerization. Gene Ontology (GO) annotations related to this gene include calcium ion binding and PDZ domain binding. An important paralog of this gene is DTNB.

UniProtKB/Swiss-Prot Summary for DTNA Gene 

May be involved in the formation and stability of synapses as well as being involved in the clustering of nicotinic acetylcholine receptors. ( DTNA_HUMAN,Q9Y4J8 )

Protein Domains for DTNA Gene

InterPro
Blocks:
  • Zn-finger, ZZ type

(5) DTNB (2p23.3) , Dystrobrevin beta.

n=DTNB&keywords=DTN

  • This gene encodes dystrobrevin beta, a component of the dystrophin-associated protein complex (DPC). The DPC consists of dystrophin and several integral and peripheral membrane proteins, including dystroglycans, sarcoglycans, syntrophins and dystrobrevin alpha and beta. The DPC localizes to the sarcolemma and its disruption is associated with various forms of muscular dystrophy. Dystrobrevin beta is thought to interact with syntrophin and the DP71 short form of dystrophin. [provided by RefSeq, Mar 2016]

GeneCards Summary for DTNB Gene 

DTNB (Dystrobrevin Beta) is a Protein Coding gene. Diseases associated with DTNB include Alacrima, Achalasia, And Impaired Intellectual Development Syndrome and Spastic Paraplegia 41, Autosomal Dominant. Among its related pathways are Non-integrin membrane-ECM interactions and Collagen chain trimerization. Gene Ontology (GO) annotations related to this gene include calcium ion binding. An important paralog of this gene is DTNA. UniProtKB/Swiss-Prot Summary for DTNB Gene Scaffolding protein that assembles DMD and SNTA1 molecules to the basal membrane of kidney cells and liver sinusoids (By similarity). May function as a repressor of the SYN1 promoter through the binding of repressor element-1 (RE-1), in turn regulates SYN1 expression and may be involved in cell proliferation regulation during the early phase of neural differentiation (PubMed:27223470). May be required for proper maturation and function of a subset of inhibitory synapses (By similarity). ( DTNB_HUMAN,O60941 )

Protein Domains for DTNB Gene

InterPro:
Blocks:
  • Zn-finger, ZZ type

(6) DYTN (2q33.3)  Dystrotelin https://www.genecards.org/cgi-bin/carddisp.pl?gene=DYTN&keywords=DYTN

NCBI Gene Summary for DYTN Gene

This gene belongs to the dystrophin superfamily, which is characterized by the presence of four EF-hand motifs and a ZZ-domain. It is a likely ortholog of the Drosophila 'discontinuous actin hexagon' gene. It is noteworthy that the coding region of this gene lacks two coding exons that are found in the mouse ortholog. Human transcripts including these two exons are subject to nonsense-mediated transcript decay (NMD). On the other hand, transcripts skipping the two coding exons are expressed at very low levels. While this gene maintains an intact CDS, it may be an evolving pseudogene. However, after a discussion about this gene within the RefSeq group, as well as in the consensus coding sequence (CCDS) collaboration, it was decided to keep it as a protein-coding gene in the RefSeq, Ensembl-GENCODE and the CCDS sets. [provided by RefSeq, Jul 2019]GeneCards Summary for DYTN Gene DYTN (Dystrotelin) is a Protein Coding gene. Diseases associated with DYTN include Hemidystonia and Leber Optic Atrophy And Dystonia. Gene Ontology (GO) annotations related to this gene include calcium ion binding. An important paralog of this gene is SPTBN5.

Protein Domains for DYTN Gene

InterPro:

(7) EP300 (22q13.2) , E1A binding protein 300, p300, KAT3B.https://www.genecards.org/Search/Keyword?queryString=EP300

  • GeneCards Symbol: EP300 2 
  • EP300 Lysine Acetyltransferase 2 3
  • Histone Acetyltransferase P300 2 3 4
  • E1A Binding Protein P300 2 3 5
  • Protein 2-Hydroxyisobutyryltransferase P300 3 4
  • Protein Propionyltransferase P300 3 4
  • Histone Crotonyltransferase P300 3 4
  • Histone Butyryltransferase P300 3 4
  • Protein Lactyltransferas P300 3 4
  • E1A-Associated Protein P300 3 4

    NCBI Gene Summary for EP300 Gene

     

    (8) HERC2 (15q13.1) , HECT and RLD domain containing E3 ubiquitin protein ligase 2. p528.

    E3 ubiquitin-protein ligase that regulates ubiquitin-dependent retention of repair proteins on damaged chromosomes. Recruited to sites of DNA damage in response to ionizing radiation (IR) and facilitates the assembly of UBE2N and RNF8 promoting DNA damage-induced formation of 'Lys-63'-linked ubiquitin chains. Acts as a mediator of binding specificity between UBE2N and RNF8. Involved in the maintenance of RNF168 levels. E3 ubiquitin-protein ligase that promotes the ubiquitination and proteasomal degradation of XPA which influences the circadian oscillation of DNA excision repair activity. By controlling the steady-state expression of the IGF1R receptor, indirectly regulates the insulin-like growth factor receptor signaling pathway (PubMed:26692333). Also modulates iron metabolism by regulating the basal turnover of FBXL5 (PubMed:24778179). ( HERC2_HUMAN,O95714 )

    https://www.genecards.org/cgi-bin/carddisp.pl?gene=HERC2&keywords=HERC2

    UniProtKB/Swiss-Prot:

     HERC2_HUMAN :
    • The ZZ-type zinc finger mediates binding to SUMO1, and at low level SUMO2.
    Domain:
    • The ZZ-type zinc finger mediates binding to SUMO1, and at low level SUMO2.
    • The RCC1 repeats are grouped into three seven-bladed beta-propeller regions.


    (9) NBR1 (17q21.31)  NBR1 autophagy cargo  receptor , KIAA0049, CA125, IAI-3B .

    • The protein encoded by this gene was originally identified as an ovarian tumor antigen monitored in ovarian cancer. The encoded protein contains a B-box/coiled-coil motif, which is present in many genes with transformation potential. It functions as a specific autophagy receptor for the selective autophagic degradation of peroxisomes by forming intracellular inclusions with ubiquitylated autophagic substrates. This gene is located on a region of chromosome 17q21.1 that is in close proximity to the BRCA1 tumor suppressor gene. Alternative splicing of this gene results in multiple transcript variants. [provided by RefSeq, Apr 2014]GeneCards Summary for NBR1 GeneNBR1 (NBR1 Autophagy Cargo Receptor) is a Protein Coding gene. Diseases associated with NBR1 include Myopathy, Myofibrillar, 9, With Early Respiratory Failure and Vici Syndrome. Among its related pathways are Selective autophagy and Perturbations to host-cell autophagy, induced by SARS-CoV-2 proteins. Gene Ontology (GO) annotations related to this gene include ubiquitin binding and mitogen-activated protein kinase binding.UniProtKB/Swiss-Prot Summary for NBR1 GeneUbiquitin-binding autophagy adapter that participates in different processes including host defense or intracellular homeostasis (PubMed:24692539, 33577621). Possesses a double function during the selective autophagy by acting as a shuttle bringing ubiquitinated proteins to autophagosomes and also by participating in the formation of protein aggregates (PubMed:24879152, 34471133). Plays a role in the regulation of the innate immune response by modulating type I interferon production and targeting ubiquitinated IRF3 for autophagic degradation (PubMed:35914352). In response to oxidative stress, promotes an increase in SQSTM1 levels, phosphorylation, and body formation by preventing its autophagic degradation (By similarity). In turn, activates the KEAP1-NRF2/NFE2L2 antioxidant pathway (By similarity). Also plays non-autophagy role by mediating the shuttle of IL-12 to late endosome for subsequent secretion (By similarity). ( NBR1_HUMAN,Q14596 )

      Protein Domains for NBR1 Gene

      InterPro:
      Blocks:
      • Ubiquitin-associated domain 
        •  
      • Zn-finger, ZZ type 
        •  
      • Octicosapeptide/Phox/Bem1p


    (10) SQSTM1 (5q35.3) , Sequestosome 1, PDB3, OS1L, p62, p60,  p62B, A170.https://www.genecards.org/cgi-bin/carddisp.pl?gene=SQSTM1&keywords=SQSTM1

    • This gene encodes a multifunctional protein that binds ubiquitin and regulates activation of the nuclear factor kappa-B (NF-kB) signaling pathway. The protein functions as a scaffolding/adaptor protein in concert with TNF receptor-associated factor 6 to mediate activation of NF-kB in response to upstream signals. Alternatively spliced transcript variants encoding either the same or different isoforms have been identified for this gene. Mutations in this gene result in sporadic and familial Paget disease of bone. [provided by RefSeq, Mar 2009]GeneCards Summary for SQSTM1 GeneSQSTM1 (Sequestosome 1) is a Protein Coding gene. Diseases associated with SQSTM1 include Paget Disease Of Bone 3 and Frontotemporal Dementia And/Or Amyotrophic Lateral Sclerosis 3. Among its related pathways are p75 NTR receptor-mediated signalling and Selective autophagy. Gene Ontology (GO) annotations related to this gene include protein homodimerization activity and protein serine/threonine kinase activity.UniProtKB/Swiss-Prot Summary for SQSTM1 GeneMolecular adapter required for selective macroautophagy (aggrephagy) by acting as a bridge between polyubiquitinated proteins and autophagosomes (PubMed:15340068, 15953362, 16286508, 17580304, 20168092, 22017874, 22622177, 24128730, 28404643, 29343546, 29507397, 31857589, 33509017, 34471133, 34893540, 35831301, 37306101, 37802024). Promotes the recruitment of ubiquitinated cargo proteins to autophagosomes via multiple domains that bridge proteins and organelles in different steps (PubMed:16286508, 20168092, 22622177, 24128730, 28404643, 29343546, 29507397, 34893540, 37802024). SQSTM1 first mediates the assembly and removal of ubiquitinated proteins by undergoing liquid-liquid phase separation upon binding to ubiquitinated proteins via its UBA domain, leading to the formation of insoluble cytoplasmic inclusions, known as p62 bodies (PubMed:15911346, 20168092, 22017874, 24128730, 29343546, 29507397, 31857589, 37802024). SQSTM1 then interacts with ATG8 family proteins on autophagosomes via its LIR motif, leading to p62 body recruitment to autophagosomes, followed by autophagic clearance of ubiquitinated proteins (PubMed:16286508, 17580304, 20168092, 22622177, 24128730, 28404643, 37802024). SQSTM1 is itself degraded along with its ubiquitinated cargos (PubMed:16286508, 17580304, 37802024). Also required to recruit ubiquitinated proteins to PML bodies in the nucleus (PubMed:20168092). Also involved in autophagy of peroxisomes (pexophagy) in response to reactive oxygen species (ROS) by acting as a bridge between ubiquitinated PEX5 receptor and autophagosomes (PubMed:26344566). Acts as an activator of the NFE2L2/NRF2 pathway via interaction with KEAP1: interaction inactivates the BCR(KEAP1) complex by sequestering the complex in inclusion bodies, promoting nuclear accumulation of NFE2L2/NRF2 and subsequent expression of cytoprotective genes (PubMed:20452972, 28380357, 33393215, 37306101). Promotes relocalization of 'Lys-63'-linked ubiquitinated STING1 to autophagosomes (PubMed:29496741). Involved in endosome organization by retaining vesicles in the perinuclear cloud: following ubiquitination by RNF26, attracts specific vesicle-associated adapters, forming a molecular bridge that restrains cognate vesicles in the perinuclear region and organizes the endosomal pathway for efficient cargo transport (PubMed:27368102, 33472082). Sequesters tensin TNS2 into cytoplasmic puncta, promoting TNS2 ubiquitination and proteasomal degradation (PubMed:25101860). May regulate the activation of NFKB1 by TNF-alpha, nerve growth factor (NGF) and interleukin-1 (PubMed:10356400, 10747026, 11244088, 12471037, 16079148, 19931284). May play a role in titin/TTN downstream signaling in muscle cells (PubMed:15802564). Adapter that mediates the interaction between TRAF6 and CYLD (By similarity). ( SQSTM_HUMAN,Q13501 )

      Protein Domains for SQSTM1 Gene

      InterPro:
      Blocks:
      • Zn-finger, ZZ type 
        •  
      • Octicosapeptide/Phox/Bem1p

    (11) TADA2B (4p16.1), Transcriptional activator 2B, MGC21874. https://www.genecards.org/cgi-bin/carddisp.pl?gene=TADA2B&keywords=TADA2B

    • TADA2B functions as a transcriptional adaptor protein that potentiates transcription through coordination of histone acetyltransferase (HAT) activity and by linking activation factors to basal transcriptional machinery (Barlev et al., 2003 [PubMed 12972612]).[supplied by OMIM, Apr 2010]GeneCards Summary for TADA2B GeneTADA2B (Transcriptional Adaptor 2B) is a Protein Coding gene. Diseases associated with TADA2B include Spinocerebellar Ataxia 7. Among its related pathways are Chromatin modifying enzymes and Deubiquitination. Gene Ontology (GO) annotations related to this gene include chromatin binding. An important paralog of this gene is TADA2A.

    UniProtKB/Swiss-Prot Summary for TADA2B Gene

    Coactivates PAX5-dependent transcription together with either SMARCA4 or GCN5L2. ( TAD2B_HUMAN,Q86TJ2 )

    Gene Families for TADA2B Gene

    HGNC:
    The Human Protein Atlas (HPA):
    • Predicted intracellular proteins
      •  
    • Transcription factors

    Protein Domains for TADA2B Gene

    InterPro:
    Blocks:
    • Zn-finger, ZZ type


    (12) UTRN (6q24.2), Utrophin (DMPL), DRP, DRP1.  3433 aa

    CBI Gene Summary for UTRN Gene

    This gene shares both structural and functional similarities with the dystrophin gene. It contains an actin-binding N-terminus, a triple coiled-coil repeat central region, and a C-terminus that consists of protein-protein interaction motifs which interact with dystroglycan protein components. The protein encoded by this gene is located at the neuromuscular synapse and myotendinous junctions, where it participates in post-synaptic membrane maintenance and acetylcholine receptor clustering. Mouse studies suggest that this gene may serve as a functional substitute for the dystrophin gene and therefore, may serve as a potential therapeutic alternative to muscular dystrophy which is caused by mutations in the dystrophin gene. Alternative splicing of the utrophin gene has been described; however, the full-length nature of these variants has not yet been determined. [provided by RefSeq, Jul 2008]

    GeneCards Summary for UTRN Gene

    UTRN (Utrophin) is a Protein Coding gene. Diseases associated with UTRN include Osteoarthritis and Muscular Dystrophy, Becker Type. Among its related pathways are Non-integrin membrane-ECM interactions and Nervous system development. Gene Ontology (GO) annotations related to this gene include calcium ion binding and actin binding. An important paralog of this gene is DMD.

    UniProtKB/Swiss-Prot Summary for UTRN Gene

    May play a role in anchoring the cytoskeleton to the plasma membrane. ( UTRN_HUMAN,P46939 )
    Quaternary structure:

    (13) KCMF1 (2p11.2), Potassium channel modulating factor 1, ZZZ1, PCMF, DEBP91.https://www.genecards.org/cgi-bin/carddisp.pl?gene=KCMF1&keywords=ZZZ1

    UniProtKB/Swiss-Prot Summary for KCMF1 Gene

    E3 ubiquitin-protein ligase which accepts ubiquitin from an E2 ubiquitin-conjugating enzyme and then transfers it to targeted substrates, promoting their degradation by the proteasome (PubMed:15581609, 25582440, 34893540, 37891180, 38297121). Together with UBR4, component of the N-end rule pathway: ubiquitinates proteins bearing specific N-terminal residues that are destabilizing according to the N-end rule, leading to their degradation (PubMed:34893540, 37891180). Does not ubiquitinate proteins that are acetylated at the N-terminus (PubMed:37891180). Together with UBR4, part of a protein quality control pathway that catalyzes ubiquitination and degradation of proteins that have been oxidized in response to reactive oxygen species (ROS): recognizes proteins with an Arg-CysO3(H) degron at the N-terminus, and mediates assembly of heterotypic 'Lys-63'-/'Lys-27'-linked branched ubiquitin chains on oxidized proteins, leading to their degradation by autophagy (PubMed:34893540). Catalytic component of the SIFI complex, a multiprotein complex required to inhibit the mitochondrial stress response after a specific stress event has been resolved: ubiquitinates and degrades (1) components of the HRI-mediated signaling of the integrated stress response, such as DELE1 and EIF2AK1/HRI, as well as (2) unimported mitochondrial precursors (PubMed:38297121). Within the SIFI complex, UBR4 initiates ubiquitin chain that are further elongated or branched by KCMF1 (PubMed:38297121). ( KCMF1_HUMAN,Q9P0J7 )

    Protein Domains for KCMF1 Gene

    InterPro:
    Blocks:
    • Zn-finger, ZZ type

    (14) ZSWIM2 (2q32.1)  Zinc Finger SWIM-type containing 3,  ZZZ2, MEX, https://www.genecards.org/cgi-bin/carddisp.pl?gene=ZSWIM2&keywords=ZZZ2

    • Predicted to enable ubiquitin protein ligase activity and zinc ion binding activity. Predicted to be involved in apoptotic process and protein polyubiquitination. Predicted to act upstream of or within positive regulation of extrinsic apoptotic signaling pathway via death domain receptors. [provided by Alliance of Genome Resources, Jul 2025] 

    ZSWIM2 (Zinc Finger SWIM-Type Containing 2) is a Protein Coding gene. Diseases associated with ZSWIM2 include Acromelic Frontonasal Dysostosis and Tylosis With Esophageal Cancer. Gene Ontology (GO) annotations related to this gene include ligase activity and obsolete protein self-association.

    E3 ubiquitin-protein ligase involved in the regulation of Fas-, DR3- and DR4-mediated apoptosis. Functions in conjunction with the UBE2D1, UBE2D3 and UBE2E1 E2 ubiquitin-conjugating enzymes.

    Protein Domains for ZSWIM2 Gene

    InterPro:
    Blocks:
    • Zn-finger, RING 
      •  
    • Zn-finger, ZZ type


    (15) ZZZ3 ( 1p31.1), Zinc Finger ZZ-type containig 3, ATAC1.

    https://www.genecards.org/cgi-bin/carddisp.pl?gene=ZZZ3&keywords=ZZZ3

    Protein Domains for ZZZ3 Gene

    InterPro:

    • Enables histone reader activity; lysine-acetylated histone binding activity; and methylated histone binding activity. Involved in several processes, including chromatin organization; regulation of cell division; and regulation of transcription by RNA polymerase II. Located in nucleolus and nucleoplasm. Part of ATAC complex. [provided by Alliance of Genome Resources, Apr 2025] 

    ZZZ3 (Zinc Finger ZZ-Type Containing 3) is a Protein Coding gene. Diseases associated with ZZZ3 include Buruli Ulcer and Combined Oxidative Phosphorylation Deficiency 20. Among its related pathways are Chromatin modifying enzymes and Epigenetic regulation by WDR5-containing histone modifying complexes. Gene Ontology (GO) annotations related to this gene include chromatin binding.

    Histone H3 reader that is required for the ATAC complex-mediated maintenance of histone acetylation and gene activation (PubMed:30217978). Component of the ATAC complex, a complex with histone acetyltransferase activity on histones H3 and H4 (PubMed:19103755). ( ZZZ3_HUMAN,Q8IYH5 )

    (16) ZZEF1 (17p13.2), Zinc Finger ZZ-type and EF-hand domain containing 1, ZZZ4. https://www.genecards.org/cgi-bin/carddisp.pl?gene=ZZEF1&keywords=ZZZ4

    • Enables histone reader activity; lysine-acetylated histone binding activity; and methylated histone binding activity. Predicted to act upstream of or within several processes, including glutamatergic synaptic transmission; regulation of peptidyl-tyrosine phosphorylation; and visual learning. Predicted to be located in cell surface; postsynapse; and presynaptic active zone. [provided by Alliance of Genome Resources, Apr 2025]

    ZZEF1 (Zinc Finger ZZ-Type And EF-Hand Domain Containing 1) is a Protein Coding gene. Gene Ontology (GO) annotations related to this gene include calcium ion binding.

    Histone H3 reader which may act as a transcriptional coactivator for KLF6 and KLF9 transcription factors. ( ZZEF1_HUMAN,O43149 )


    (17) MIB2 (1p36.33) , Mind bomb E3 ubiquitin ligase 2, ZZZ5, ZZANK1, Skeletrophin.https://www.genecards.org/cgi-bin/carddisp.pl?gene=MIB2&keywords=ZZANK1

    • The protein encoded by this gene is an E3 ubiquitin protein ligase that mediates ubiquitination of proteins in the Notch signaling pathway. The encoded protein may be a suppressor of melanoma invasion. [provided by RefSeq, Mar 2017]

    MIB2 (MIB E3 Ubiquitin Protein Ligase 2) is a Protein Coding gene. Diseases associated with MIB2 include Left Ventricular Noncompaction and Optic Nerve Hypoplasia, Bilateral. Among its related pathways are Constitutive Signaling by NOTCH1 HD+PEST Domain Mutants and NOTCH2 Activation and Transmission of Signal to the Nucleus. Gene Ontology (GO) annotations related to this gene include actin binding and ubiquitin-protein transferase activity. An important paralog of this gene is MIB1.

    E3 ubiquitin-protein ligase that mediates ubiquitination of Delta receptors, which act as ligands of Notch proteins. Positively regulates the Delta-mediated Notch signaling by ubiquitinating the intracellular domain of Delta, leading to endocytosis of Delta receptors

    Gene Families for MIB2 Gene

    HGNC:
    The Human Protein Atlas (HPA):
    • Enzymes
      •  
    • Metabolic proteins
      •  
    • Predicted intracellular proteins

    Protein Domains for MIB2 Gene

    InterPro:
    Blocks:
    • Zn-finger, RING 
      •  
    • Zn-finger, ZZ type

    (18) MIB1 (18q11.2), Mind bomb E3 ubiquitin  protein ligase 1, ZZZ6, DIP-1, MIB, ZZANK2. https://www.genecards.org/cgi-bin/carddisp.pl?gene=MIB1&keywords=ZZZ6

    Gene Families for MIB2 Gene

    HGNC:
    The Human Protein Atlas (HPA):
    • Enzymes
      •  
    • Metabolic proteins
      •  
    • Predicted intracellular proteins

    Protein Domains for MIB2 Gene

    InterPro:
    Blocks:
    • Zn-finger, RING 
      •  
    • Zn-finger, ZZ type



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    Jos nyt tähän luetteloon uskaltaa ehdotata lisää, niin se Duodecimartikkelin  molekyyli CPEB1 on ainakin yhdessä artikkelissa netissä mainittu  CPEB1-ZZ nimellä ja siinä on C-terminaalin 

    binukleaarista sinkkiä sisältävä kohta.  Otan siitä erikseen  lähteitä.  Luetetlen se kutienin ZZ_ tyyppisiin, vaikka siinä  mainitaan havaittavan muitakin domeeneja, kuten monessa ZNF- proteiinissa voi olla usea erilaisen  alatyyppidomeeni.  Sen takia  CPEB  geenin ja proteiinin voinee löytää jostain muusta  ZNF-ryhmistä myös. ( Ei voi muuten)


    Tällaisesta esimerkki on tuossa luettelossa  numero 14. tuo ZZZ2. Se maintiaan alatyypiksi ZSWIM myös  eli "Zinc Finger protein SWIM-type" ryhmäläisenä, eikä vain  Zinc finger protein ZZ_ type" ryhmäläisenä. Otan seuraavaksi Merkelin et al.  artikkelin suomennoksen, mikä osoittaa,  miten paljon domeeneja  havaitaan CDEB1- proteiinista.

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