ID:UBXN1_HUMAN DESCRIPTION: RecName: Full=UBX domain-containing protein 1; AltName: Full=SAPK substrate protein 1; AltName: Full=UBA/UBX 33.3 kDa protein; FUNCTION: Ubiquitin-binding protein that interacts with the BRCA1- BARD1 heterodimer, and regulates its activity. Specifically binds 'Lys-6'-linked polyubiquitin chains. Interaction with autoubiquitinated BRCA1, leads to inhibit the E3 ubiquitin-protein ligase activity of the BRCA1-BARD1 heterodimer. Component of a complex required to couple deglycosylation and proteasome-mediated degradation of misfolded proteins in the endoplasmic reticulum that are retrotranslocated in the cytosol. SUBUNIT: Component of a complex required to couple retrotranslocation, ubiquitination and deglycosylation composed of NGLY1, SAKS1, AMFR, VCP and RAD23B. Interacts with HOMER2 (By similarity). Interacts directly with VCP. Interacts with BRCA1 and BARD1; interaction takes place when BRCA1 is not autoubiquitinated but is strongly enhanced in the presence of autoubiquitinated BRCA1. SUBCELLULAR LOCATION: Cytoplasm (By similarity). DOMAIN: The UBA domain specifically recognizes and binds 'Lys-6'- linked polyubiquitin chains. SIMILARITY: Contains 1 UBA domain. SIMILARITY: Contains 1 UBX domain.
The RNAfold program from the Vienna RNA Package is used to perform the secondary structure predictions and folding calculations. The estimated folding energy is in kcal/mol. The more negative the energy, the more secondary structure the RNA is likely to have.
ModBase Predicted Comparative 3D Structure on Q04323
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Orthologous Genes in Other Species
Orthologies between human, mouse, and rat are computed by taking the best BLASTP hit, and filtering out non-syntenic hits. For more distant species reciprocal-best BLASTP hits are used. Note that the absence of an ortholog in the table below may reflect incomplete annotations in the other species rather than a true absence of the orthologous gene.
Biological Process: GO:0006457 protein folding GO:0031397 negative regulation of protein ubiquitination GO:0032435 negative regulation of proteasomal ubiquitin-dependent protein catabolic process GO:0043161 proteasome-mediated ubiquitin-dependent protein catabolic process GO:1903094 negative regulation of protein K48-linked deubiquitination GO:1904293 negative regulation of ERAD pathway GO:2000157 negative regulation of ubiquitin-specific protease activity
BC001372 - Homo sapiens UBX domain protein 1, mRNA (cDNA clone MGC:2274 IMAGE:3051213), complete cds. BC000902 - Homo sapiens UBX domain protein 1, mRNA (cDNA clone MGC:5321 IMAGE:2900493), complete cds. BC032689 - Homo sapiens UBX domain protein 1, mRNA (cDNA clone IMAGE:5241425), complete cds. BC040129 - Homo sapiens UBX domain protein 1, mRNA (cDNA clone MGC:48785 IMAGE:6065505), complete cds. M68864 - Human ORF mRNA, complete cds. AK054832 - Homo sapiens cDNA FLJ30270 fis, clone BRACE2002661, highly similar to UBA/UBX 33.3 kDa protein. AK303471 - Homo sapiens cDNA FLJ53007 complete cds, highly similar to UBA/UBX 33.3 kDa protein. JD380452 - Sequence 361476 from Patent EP1572962. GQ129328 - Synthetic construct Homo sapiens clone HAIB:100068582; DKFZo008D0535 SAPK substrate protein 1 protein (LOC51035) gene, complete cds. KJ902543 - Synthetic construct Homo sapiens clone ccsbBroadEn_11937 UBXN1 gene, encodes complete protein. JD377105 - Sequence 358129 from Patent EP1572962. HQ448734 - Synthetic construct Homo sapiens clone IMAGE:100072180; CCSB006778_02 SAPK substrate protein 1 (LOC51035) gene, encodes complete protein. KJ898807 - Synthetic construct Homo sapiens clone ccsbBroadEn_08201 UBXN1 gene, encodes complete protein. JD022124 - Sequence 3148 from Patent EP1572962. JD031346 - Sequence 12370 from Patent EP1572962. AK316012 - Homo sapiens cDNA, FLJ78911 complete cds, highly similar to UBA/UBX 33.3 kDa protein. AK297856 - Homo sapiens cDNA FLJ58850 complete cds, highly similar to UBA/UBX 33.3 kDa protein. AK300542 - Homo sapiens cDNA FLJ58945 complete cds, highly similar to UBA/UBX 33.3 kDa protein. CU674676 - Synthetic construct Homo sapiens gateway clone IMAGE:100017009 5' read LOC51035 mRNA. JD129557 - Sequence 110581 from Patent EP1572962. JD197409 - Sequence 178433 from Patent EP1572962.
Biochemical and Signaling Pathways
Reactome (by CSHL, EBI, and GO)
Protein Q04323 (Reactome details) participates in the following event(s):
R-HSA-8850594 Deglycosylation complex hydrolyses N-glycans from unfolded glycoproteins R-HSA-532668 N-glycan trimming in the ER and Calnexin/Calreticulin cycle R-HSA-446203 Asparagine N-linked glycosylation R-HSA-597592 Post-translational protein modification R-HSA-392499 Metabolism of proteins
US Server
Sequence and Links to Tools and Databases 
Common Gene Haplotype Alleles 
