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ERO1-like protein alpha; ERO1-L; ERO1-L-alpha; endoplasmic oxidoreductin-1-like protein; oxidoreductin-1-L-alpha (ERO1L)

Function: 1) oxidoreductase 2) provides oxidizing equivalents in the endoplasmic reticulum (ER) for the formation of disulfide bonds 3) directly oxidizes P4HB/PDI isomerase via direct disulfide exchange 4) does NOT act as a direct oxidant of folding substrate, but relies on P4HB/PDI to transfer oxidizing equivalent 5) does not oxidize all PDI related proteins a) can discriminate between PDI & related proteins. b) associates with TXNDC4 but not with GRP54 6) reoxidation involves electron transfer to O2 via FAD 7) association with TXNDC4 retains ERO1LB in the ER 8) involved in folding of immunoglobulins Cofactor: FAD Structure: - monomer & homodimer - belongs to the EROs family Compartment: endoplasmic reticulum, lumenal side Expression: - widely expressed at low level - expressed at high level in upper digestive tract esophagus > stomach, duodenum - induced by hypoxia Pathology: - release of unfolded cholera toxin (retrotranslocation) from reduced P4HB/PDI during infection by V.cholerae

General

glycoprotein oxidoreductase

Properties

SIZE: entity length = 468 aa MW = 54 kD COMPARTMENT: endoplasmic reticulum MOTIF: signal sequence {1-23} cysteine residue {C35} MODIFICATION: cysteine residue {C46} cysteine residue {C37} MODIFICATION: cysteine residue {C48} cysteine residue {C46} MODIFICATION: cysteine residue {C35} cysteine residue {C48} MODIFICATION: cysteine residue {C37} cysteine residue {C85} MODIFICATION: cysteine residue {C391} cysteine residue {C94} MODIFICATION: cysteine residue {C131} cysteine residue {C94} MODIFICATION: cysteine residue {C99} cysteine residue {C99} MODIFICATION: cysteine residue {C94} cysteine residue {C99} MODIFICATION: cysteine residue {C104} cysteine residue {C104} MODIFICATION: cysteine residue {C99} cysteine residue {C131} MODIFICATION: cysteine residue {C94} binding site SITE: 187-187 FOR-BINDING-OF: FAD binding site SITE: 189-189 FOR-BINDING-OF: FAD binding site SITE: 200-200 FOR-BINDING-OF: FAD cysteine residue {C208} MODIFICATION: cysteine residue {C241} cysteine residue {C241} MODIFICATION: cysteine residue {C208} binding site SITE: 252-252 FOR-BINDING-OF: FAD binding site SITE: 255-255 FOR-BINDING-OF: FAD N-glycosylation site {N280} binding site SITE: 287-287 FOR-BINDING-OF: FAD N-glycosylation site {N384} cysteine residue {C391} MODIFICATION: cysteine residue {C85} cysteine residue {C394} MODIFICATION: cysteine residue {C397} cysteine residue {C397} MODIFICATION: cysteine residue {C394}

Database Correlations

UniProt Q96HE7 Pfam PF04137 Entrez Gene 30001 Kegg hsa:30001

References

UniProt :accession Q96HE7