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

More general terms

• hydroxylase; monooxygenase
• dioxygenase
• phosphoprotein
• glycoprotein
• membrane protein

Database

• Entrez gene: http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&cmd=Retrieve&dopt=Graphics&list_uids=444
• Kegg: http://www.genome.jp/dbget-bin/www_bget?hsa:444
• OMIM: https://mirror.omim.org/entry/600582
• UniProt: http://www.uniprot.org/uniprot/Q12797.html