MitoProteome Database

MT001310

UniProt Annotations

Entry Information
Gene Namechondroitin polymerizing factor
Protein EntryCHSS2_HUMAN
UniProt IDQ8IZ52
SpeciesHuman
Comments
Comment typeDescription
Alternative Products Event=Alternative splicing; Named isoforms=3; Name=1; IsoId=Q8IZ52-1; Sequence=Displayed; Name=2; Synonyms=Klokin1; IsoId=Q8IZ52-2; Sequence=VSP_053433; Name=3; Synonyms=ChPF(D996); IsoId=Q8IZ52-4; Sequence=VSP_053434;
Catalytic Activity UDP-alpha-D-glucuronate + N-acetyl-beta-D- galactosaminyl-(1->4)-beta-D-glucuronosyl-proteoglycan = UDP + beta-D-glucuronosyl-(1->3)-N-acetyl-beta-D-galactosaminyl-(1->4)- beta-D-glucuronosyl-proteoglycan.
Catalytic Activity UDP-N-acetyl-alpha-D-galactosamine + beta-D- glucuronosyl-(1->3)-N-acetyl-beta-D-galactosaminyl-proteoglycan = UDP + N-acetyl-beta-D-galactosaminyl-(1->4)-beta-D-glucuronosyl- (1->3)-N-acetyl-beta-D-galactosaminyl-proteoglycan.
Cofactor Name=Mn(2+); Xref=ChEBI:CHEBI:29035; Evidence={ECO:0000269|PubMed:12761225}; Name=Co(2+); Xref=ChEBI:CHEBI:48828; Evidence={ECO:0000269|PubMed:12761225}; Note=Highest activities are measured with Mn(2+). Can also utilize Co(2+). {ECO:0000269|PubMed:12761225};
Function Has both beta-1,3-glucuronic acid and beta-1,4-N- acetylgalactosamine transferase activity. Transfers glucuronic acid (GlcUA) from UDP-GlcUA and N-acetylgalactosamine (GalNAc) from UDP-GalNAc to the non-reducing end of the elongating chondroitin polymer. Isoform 2 may facilitate PARK2 transport into the mitochondria. In collaboration with PARK2, isoform 2 may enhance cell viability and protect cells from oxidative stress. {ECO:0000269|PubMed:12761225}.
Interaction O60260:PARK2; NbExp=5; IntAct=EBI-9029620, EBI-716346;
Similarity Belongs to the chondroitin N- acetylgalactosaminyltransferase family. {ECO:0000305}.
Subcellular Location Isoform 1: Golgi apparatus, Golgi stack membrane {ECO:0000305}; Single-pass type II membrane protein {ECO:0000305}. Cytoplasm, cytosol.
Subcellular Location Isoform 2: Mitochondrion matrix {ECO:0000305}.
Subcellular Location Isoform 3: Cytoplasm, cytosol. Mitochondrion.
Subunit Binds CHSY1. Isoform 1, isoform 2 and isoform 3 interact with PARK2.
Tissue Specificity Ubiquitous. Highly expressed in pancreas, ovary, brain, heart, skeletal muscle, colon, kidney, liver, stomach, spleen and placenta. Isoform 3 is also ubiquitous. Isoform 2 is expressed in brain, spleen, ovary, testis, lung and peripheral mononuclear cells. {ECO:0000269|PubMed:12716890, ECO:0000269|PubMed:12761225, ECO:0000269|PubMed:22082830}.
Web Resource Name=GGDB; Note=GlycoGene database; URL="http://jcggdb.jp/rcmg/ggdb/Homolog?cat=symbol&symbol=CHPF";