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aspartyl/asparaginyl beta-hydroxylase; aspartate beta-hydroxylase; ASP beta-hydroxylase; peptide-aspartate beta-dioxygenase; junctin/junctate (ASPH, HAAH, BAH)
Function:
- specifically hydroxylates an Asp or Asn in certain epidermal growth factor-like domains (EGF domains) of a number of proteins
peptide L-aspartate + 2-oxoglutarate + O2 = peptide 3-hydroxy-L-aspartate + succinate + CO2
Cofactor: iron
Structure:
- monomer (putative)
- belongs to the aspartyl/asparaginyl beta-hydroxylase family
- contains 4 TPR repeats
Compartment:
- isoform 1: endoplasmic reticulum membrane
- single-pass type 2 membrane protein
Alternative splicing:
- named isoforms=4;
- junctate, junctin-1, junctin-2
Expression:
- isoform 1 is detected in all tissues tested
General
dioxygenase
glycoprotein
hydroxylase; monooxygenase
membrane protein
phosphoprotein
Properties
SIZE: entity length = 758 aa
MW = 86 kD
COMPARTMENT: endoplasmic reticulum
MOTIF: serine-rich region {9-29}
MOTIF: serine residue (SEVERAL)
Ser phosphorylation site {S29}
transmembrane domain {54-74}
glutamate-rich region {111-312}
MOTIF: glutamate residue (SEVERAL)
lysine-rich region {323-332}
MOTIF: lysine residue (SEVERAL)
tetratricopeptide repeat {341-374}
N-glycosylation site {N452}
tetratricopeptide repeat {454-487}
tetratricopeptide repeat {489-521}
tetratricopeptide repeat {525-557}
N-glycosylation site {N706}
Database Correlations
OMIM 600582
UniProt Q12797
PFAM correlations
Entrez Gene 444
Kegg hsa:444
ENZYME 1.14.11.16
References
UniProt :accession Q12797