| 1306167 | Stub1 | STIP1 homology and U-box containing protein 1 | ENCODES a protein that exhibits protein-folding chaperone binding; ubiquitin protein ligase activity; enzyme binding (ortholog); INVOLVED IN cellular response to heat; cellular response to hypoxia; chaperone-mediated autophagy; PARTICIPATES IN Endoplasmic Reticulum-associated degradation pathway; ub iquitin/proteasome degradation pathway; ASSOCIATED WITH autosomal recessive spinocerebellar ataxia 16 (ortholog); cerebellar ataxia type 48 (ortholog); genetic disease (ortholog); FOUND IN cytoplasm; mitochondrion; nucleus; INTERACTS WITH 2,3,7,8-tetrachlorodibenzodioxine; 2,4-dinitrotoluene; 3-chloropropane-1,2-diol | 10 | 15355278 | 15357559 | Rat | 274 | symbol , PhenoGen | gene, protein-coding, VALIDATED [RefSeq] |
| 1314629 | STUB1 | STIP1 homology and U-box containing protein 1 | 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 spinocer ebellar 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] | 16 | 680410 | 682801 | Human | 368 | symbol , COSMIC , Human Proteome Map | gene, protein-coding, REVIEWED [RefSeq] |
| 1314630 | Stub1 | STIP1 homology and U-Box containing protein 1 | Enables protein homodimerization activity; ubiquitin protein ligase binding activity; and ubiquitin-ubiquitin ligase activity. Involved in several processes, including negative regulation of peroxisome proliferator activated receptor signaling pathway; positive regulation of ubiquitin-protein transf erase activity; and proteolysis involved in protein catabolic process. Acts upstream of or within protein polyubiquitination. Located in Z disc. Is expressed in tooth. Used to study autosomal recessive spinocerebellar ataxia 16. Human ortholog(s) of this gene implicated in autosomal recessive spinocerebellar ataxia 16 and cerebellar ataxia type 48. Orthologous to human STUB1 (STIP1 homology and U-box containing protein 1). [provided by Alliance of Genome Resources, Apr 2025] | 17 | 26049608 | 26051893 | Mouse | 330 | symbol , PhenoGen , description | gene, protein-coding, VALIDATED [RefSeq] |
| 9036291 | Stub1 | STIP1 homology and U-box containing protein 1 | ENCODES a protein that exhibits enzyme binding (ortholog); G protein-coupled receptor binding (ortholog); heat shock protein binding (ortholog); INVOLVED IN cellular response to heat (ortholog); cellular response to hypoxia (ortholog); cellular response to misfolded protein (ortholog); ASSOCIATED WI TH autosomal recessive spinocerebellar ataxia 16 (ortholog); cerebellar ataxia type 48 (ortholog); genetic disease (ortholog); FOUND IN cytoplasm (ortholog); cytosol (ortholog); endoplasmic reticulum (ortholog) | NW_004955442 | 16521130 | 16523733 | Chinchilla | 150 | symbol | gene, protein-coding, MODEL [RefSeq] |
| 12325293 | STUB1 | STIP1 homology and U-box containing protein 1 | ENCODES a protein that exhibits enzyme binding (inferred); G protein-coupled receptor binding (inferred); heat shock protein binding (inferred); INVOLVED IN cellular response to heat (ortholog); cellular response to hypoxia (ortholog); chaperone-mediated autophagy (ortholog); ASSOCIATED WITH autosom al recessive spinocerebellar ataxia 16 (ortholog); cerebellar ataxia type 48 (ortholog); genetic disease (ortholog); FOUND IN cytoplasm (inferred); cytosol (inferred); endoplasmic reticulum (inferred) | 6 | 39893492 | 39895385 | Dog | 138 | symbol | gene, protein-coding, MODEL [RefSeq] |
| 12452606 | Stub1 | STIP1 homology and U-box containing protein 1 | ENCODES a protein that exhibits enzyme binding (inferred); G protein-coupled receptor binding (inferred); heat shock protein binding (inferred); INVOLVED IN cellular response to heat (ortholog); cellular response to hypoxia (ortholog); chaperone-mediated autophagy (ortholog); ASSOCIATED WITH autosom al recessive spinocerebellar ataxia 16 (ortholog); cerebellar ataxia type 48 (ortholog); genetic disease (ortholog); FOUND IN cytoplasm (inferred); cytosol (inferred); endoplasmic reticulum (inferred) | NW_004936501 | 328767 | 330963 | Squirrel | 138 | symbol | gene, protein-coding, MODEL [RefSeq] |
| 18477711 | STUB1 | STIP1 homology and U-box containing protein 1 | ENCODES a protein that exhibits enzyme binding (inferred); G protein-coupled receptor binding (inferred); heat shock protein binding (inferred); INVOLVED IN cellular response to heat (ortholog); cellular response to hypoxia (ortholog); chaperone-mediated autophagy (ortholog); ASSOCIATED WITH autosom al recessive spinocerebellar ataxia 16 (ortholog); cerebellar ataxia type 48 (ortholog); genetic disease (ortholog); FOUND IN cytoplasm (inferred); cytosol (inferred); endoplasmic reticulum (inferred) | | | | Green Monkey | 138 | symbol | gene, protein-coding, MODEL [RefSeq] |
| 18922165 | Stub1 | STIP1 homology and U-box containing protein 1 | ENCODES a protein that exhibits enzyme binding (ortholog); G protein-coupled receptor binding (ortholog); heat shock protein binding (ortholog); INVOLVED IN cellular response to heat (ortholog); cellular response to hypoxia (ortholog); chaperone-mediated autophagy (ortholog); ASSOCIATED WITH autosom al recessive spinocerebellar ataxia 16 (ortholog); cerebellar ataxia type 48 (ortholog); genetic disease (ortholog); FOUND IN cytosol (ortholog); endoplasmic reticulum (ortholog); nuclear inclusion body (ortholog) | | | | Naked Mole-Rat | 135 | symbol | gene, protein-coding, MODEL [RefSeq] |
| 626090587 | Stub1 | STIP1 homology and U-box containing protein 1 | ENCODES a protein that exhibits enzyme binding (ortholog); G protein-coupled receptor binding (ortholog); heat shock protein binding (ortholog); INVOLVED IN cellular response to heat (ortholog); cellular response to hypoxia (ortholog); cellular response to misfolded protein (ortholog); ASSOCIATED WI TH autosomal recessive spinocerebellar ataxia 16 (ortholog); cerebellar ataxia type 48 (ortholog); genetic disease (ortholog); FOUND IN cytoplasm (ortholog); cytosol (ortholog); endoplasmic reticulum (ortholog) | | | | Black Rat | 150 | symbol | gene, protein-coding, MODEL [RefSeq] |
| 14021200 | STUB1 | STIP1 homology and U-box containing protein 1 | ENCODES a protein that exhibits enzyme binding (inferred); G protein-coupled receptor binding (inferred); heat shock protein binding (inferred); INVOLVED IN cellular response to heat (ortholog); cellular response to hypoxia (ortholog); chaperone-mediated autophagy (ortholog); ASSOCIATED WITH autosom al recessive spinocerebellar ataxia 16 (ortholog); cerebellar ataxia type 48 (ortholog); genetic disease (ortholog); FOUND IN cytoplasm (inferred); cytosol (inferred); endoplasmic reticulum (inferred) | | | | Pig | 138 | symbol | gene, protein-coding, MODEL [RefSeq] |
| 155238473 | stub1 | | | | | | Tropical Clawed Frog | | symbol | gene, null |
| 12048029 | STUB1 | STIP1 homology and U-box containing protein 1 | ENCODES a protein that exhibits enzyme binding (inferred); G protein-coupled receptor binding (inferred); heat shock protein binding (inferred); INVOLVED IN cellular response to heat (ortholog); cellular response to hypoxia (ortholog); chaperone-mediated autophagy (ortholog); ASSOCIATED WITH autosom al recessive spinocerebellar ataxia 16 (ortholog); cerebellar ataxia type 48 (ortholog); genetic disease (ortholog); FOUND IN cytoplasm (inferred); cytosol (inferred); endoplasmic reticulum (inferred) | | | | Bonobo | 138 | symbol | gene, protein-coding, MODEL [RefSeq] |
| 14382652 | STUB1 | | | | | | Fly | | symbol | gene, null |
| 155238472 | stub1.L | | | | | | African Clawed Frog | | symbol | gene, null |
| 155244450 | stub1.S | | | | | | African Clawed Frog | | symbol | gene, null |
| 14974606 | STUB1-DT | STUB1 divergent transcript | INTERACTS WITH aristolochic acid A | 16 | 678518 | 679777 | Human | 1 | symbol , COSMIC , name , Human Proteome Map | gene, pseudo, VALIDATED [RefSeq] |
| 15091074 | STUB1P1 | STUB1 pseudogene 1 | | 2 | 177177697 | 177178458 | Human | | symbol , GTEx , COSMIC , name , Human Proteome Map | gene, pseudo, INFERRED [RefSeq] |
| 16553428 | AC079305.3 | STIP1 homology and U-box containing protein 1 (STUB1) pseudogene | | | | | Human | | name | gene, processed_pseudogene |