RING
-tyyppisten E3 -ligaasien alaryhmä U-BOX tyyppiset E3 eli
(1) UBOX1, CHIP,
(2) UBOX2, E4A ja
E4
ubikitiiniligaasi on E1-, E2-, E3-koordinaattori
(3) UBOX3, E4B .
Lisäys 10.11.2025: UBOX4.
(1) UBOX1 ( 16p13.3), E3 ubiquitin-proteine ligase CHIP, STUB1
STUB1,
STIP1
homology and U-box containing protein 1 [ Homo
sapiens (human)
]
- Also known as
- CHIP; SCA48; UBOX1; SCAR16; HSPABP2; NY-CO-7; SDCCAG7
- Summary
- This gene encodes a protein containing tetratricopeptide repeat and a U-box that functions as a ubiquitin ligase/cochaperone. The encoded protein binds to and ubiquitinates shock cognate 71 kDa protein (Hspa8) and DNA polymerase beta (Polb), among other targets. Mutations in this gene cause spinocerebellar ataxia, autosomal recessive 16. Alternative splicing results in multiple transcript variants. There is a pseudogene for this gene on chromosome 2. [provided by RefSeq, Jun 2014]
- Expression Ubiquitous expression in fat (RPKM 42.9), kidney (RPKM 39.1) and 25 other tissues See more Orthologs mouse all
-
- Preferred Names
- E3 ubiquitin-protein ligase CHIP
- Names
- CLL-associated antigen KW-8
- RING-type E3 ubiquitin transferase CHIP
- STIP1 homology and U-box containing protein 1, E3 ubiquitin protein ligase
- antigen NY-CO-7
- carboxy terminus of Hsp70-interacting protein
- heat shock protein A binding protein 2 (c-terminal)
- serologically defined colon cancer antigen 7
- Conserved Domains (3) summary
-
sd00006
Location:21 → 51 - TPR; TPR repeat [structural motif]
-
pfam12895
Location:3 → 48 - ANAPC3; Anaphase-promoting complex, cyclosome, subunit 3
-
cd16654
Location:155 → 221 - RING-Ubox_CHIP; U-box domain, a modified RING finger, found in carboxyl terminus of HSP70-interacting protein (CHIP) and similar proteins
(2) UBOX2 (11q23.2) , UBE4A
UBE4A ubiquitination factor E4A [ Homo sapiens (human) ]
E4 ubikitiiniligaasi eli U-box ubikitiiniligaasiperhetta, joka osallistuu jmultiubikitiiniketjujen koostamiseen ja on tärkeä kromosomiaineksen tiivistymisessä ja erkanemisissa sekuriinin poluyubikitinaation avulla. Autovasta-aineet ovat merkkareita sklerodermasta ja Crohnin taudista. Muita nimiä E4, UFD2 ja UBOX2. Tätä ilmenee aivossa ja umpisuolenlisäkkeessä.- Also known as E4A; ( UFD2 holomog yest); UBOX2
- Summary This gene encodes a member of the U-box ubiquitin ligase family. The encoded protein is involved in multiubiquitin chain assembly and plays a critical role in chromosome condensation and separation through the polyubiquitination of securin. Autoantibodies against the encoded protein may be markers for scleroderma and Crohn's disease. A pseudogene of this gene is located on the long arm of chromosome 3. Alternatively spliced transcript variants encoding multiple isoforms have been observed for this gene. [provided by RefSeq, Feb 2011]
- Expression Ubiquitous expression in brain (RPKM 15.5), appendix (RPKM 14.1) and 25 other tissues See more Orthologs mouse all
- Preferred Names
- ubiquitin conjugation factor E4 A
- Names
- RING-type E3 ubiquitin transferase E4 A
- ubiquitination factor E4A (UFD2 homolog, yeast)
- Conserved Domains (2) summary
-
pfam10408
Location:330 → 971 - Ufd2P_core; Ubiquitin elongating factor core . This is the most conserved part of the core region of Ufd2P ubiquitin elongating factor or E4, running from helix alpha-11 to alpha-38. It consists of 31 helices of variable length connected by loops of variable size forming a compact unit; the helical packing pattern of the compact unit consists of five structural repeats that resemble tandem Armadillo (ARM) repeats. This domain is involved in ubiquitination as it binds Cdc48p and escorts ubiquitinated proteins from Cdc48p to the proteasome for degradation. The core is structurally similar to the nuclear transporter protein importin-alpha. The core is associated with the U-box at the C-terminus, pfam04564, which has ligase activity
-
cd16657
Location:990 → 1059 - RING-Ubox_UBE4A;
- U-box domain, a modified RING finger, found in ubiquitin conjugation factor E4 A (UBE4A) and similar proteins
- The family includes yeast ubiquitin fusion degradation protein 2 (UFD2p) and its mammalian homolog, UBE4A. Yeast UFD2p, also known as ubiquitin conjugation factor E4 or UB fusion protein 2, is a polyubiquitin chain conjugation factor (E4) in the ubiquitin fusion degradation (UFD) pathway which catalyzes elongation of the ubiquitin chain through Lys48 linkage. It binds to substrates conjugated with one to three ubiquitin molecules and catalyzes the addition of further ubiquitin moieties in the presence of ubiquitin-activating enzyme (E1), ubiquitin-conjugating enzyme (E2) and ubiquitin ligase (E3), yielding
(3) UBOX3 (1p36.22), UBE4B
Tämäkin proteiini on niitä
RING-tyyppisten E3-ubikitiinitransferaasi E4 B tyyppinen eli
ubikitinaatiofaktori E4B, joilla on se U-box. Tämä on UBOX3.
Lyhennyksiä kertyy monta
tällekin.
https://www.ncbi.nlm.nih.gov/gene/10277
https://www.ncbi.nlm.nih.gov/gene/10277
UBE4B ubiquitination factor E4B [ Homo sapiens (human) ]
- Also known as
- E4; UFD2; HDNB1; UBOX3; UFD2A
- Summary
- The modification of proteins with ubiquitin is an important cellular mechanism for targeting abnormal or short-lived proteins for degradation. Ubiquitination involves at least three classes of enzymes: ubiquitin-activating enzymes, or E1s, ubiquitin-conjugating enzymes, or E2s, and ubiquitin-protein ligases, or E3s. This gene encodes an additional conjugation factor, E4, which is involved in multiubiquitin chain assembly. This gene is also the strongest candidate in the neuroblastoma tumor suppressor genes. Alternatively spliced transcript variants encoding distinct isoforms have been found for this gene. [provided by RefSeq, Jul 2008]
- Expression Ubiquitous expression in thyroid (RPKM 12.1), skin (RPKM 10.9) and 25 other tissues See more
- Orthologs mouse all
- Preferred Names
- ubiquitin conjugation factor E4 B
- Names
- RING-type E3 ubiquitin transferase E4 B
- UFD2A-III/UBE4B-III splice isoform
- homologous to yeast UFD2
- homozygously deleted in neuroblastoma-1 (HDNB1)
- ubiquitin-fusion degradation protein 2 (UFD2)
- ubiquitination factor E4B (UFD2 homolog, yeast)
Yhteenvetona U-box tyyppisistä E3 ligaaseista ”E4 ”
https://www.genecards.org/cgi-bin/carddisp.pl?gene=PRPF19&keywords=UBOX4UBOX4 Asetan tämän erikseen uusimmaksi artikkeliksi 2025. (10.11.2025
Aliases for PRPF19 Gene
- Pre-MRNA-Processing Factor 19 3 4
- Nuclear Matrix Protein 200 3 4
- Senescence Evasion Factor 3 4
- PRP19/PSO4 Homolog 3 4
- Psoralen 4 2 3
- PRP19/PSO4 Pre-MRNA Processing Factor 19 Homolog (S. Cerevisiae) 2
- PRP19/PSO4 Pre-MRNA Processing Factor 19 Homolog 3
- PRP19/PSO4 Homolog (S. Cerevisiae) 2
- EC 2.3.2.27 4
- HPso4 4
