QSOX1

QSOX1
Available structures
PDBOrtholog search: PDBe RCSB
Identifiers
AliasesQSOX1, Q6, QSCN6, quiescin sulfhydryl oxidase 1
External IDsOMIM: 603120; MGI: 1330818; HomoloGene: 37690; GeneCards: QSOX1; OMA:QSOX1 - orthologs
Orthologs
SpeciesHumanMouse
Entrez

5768

104009

Ensembl

ENSG00000116260

ENSMUSG00000033684

UniProt

O00391

Q8BND5

RefSeq (mRNA)

NM_002826
NM_001004128

NM_001024945
NM_023268

RefSeq (protein)

NP_001004128
NP_002817

NP_001020116
NP_075757

Location (UCSC)Chr 1: 180.15 – 180.2 MbChr 1: 155.65 – 155.69 Mb
PubMed search
Wikidata
View/Edit HumanView/Edit Mouse

Quiescin sulfhydryl oxidase 1 is an enzyme that in humans is encoded by the QSOX1 gene.

This gene encodes an enzyme that is localized primarily to the Golgi apparatus and secreted fluids. QSOX1 is a multi-domain disulfide catalyst. Unlike other disulfide catalysts, QSOX1 can both generate disulfides de novo and catalyze dithiol/disulfide exchange. The de novo disulfide bond formation is catalyzed by the ERV1 domain that contains a FAD co-factor that allows depositions of electrons onto the terminal electron acceptor, typically molecular oxygen. The dithiol/disulfide exchange is catalyzed by the thioredoxin domain. The two domains are linked together by a flexible linker that allows the thioredoxin domain to first interact with the substrate protein and then regenerate by ERV1 domain.

QSOX1 gene expression is induced as fibroblasts begin to exit the proliferative cycle and enter quiescence, suggesting that this gene plays an important role in growth regulation. In fibroblasts QSOX1 is required for normal incorporation of laminin into the extracellular matrix, and thereby for normal cell-cell adhesion and cell migration.

Two transcript variants encoding two different isoforms have been found for this gene. The two isoforms have different tissue distribution and in addition isoform 1 has a transmembrane helix in the carboxy terminal while isoform 2 is a secreted soluble protein.