Tgds
entrez
76355
canonical
uniprot
Q8VDR7
canonical
ensembl
ENSMUSG00000022130
resolver
ensp
ENSMUSP00000022727
resolver
enst
ENSMUST00000022727
resolver
genesymbol
Tgds
resolver
uniprot
Q3TMF9
resolver
uniprot
Q3U4A6
resolver
uniprot_entry
Q3TMF9_MOUSE
resolver
uniprot_entry
TGDS_MOUSE
resolver
uniprot_sec
Q3U4A6
resolver
amino_acid_sequence
MSAASREERSGPPGSFAKRVLVTGGAGFIASHVIVSLVEDYPDYMIVNLDKLDYCASLKNLEPVSNKQNYKFIQGDICDSHFVKLLFEVEKIDIVLHFAAQTHVDLSFVRAFEFTYVNVYGTHVLVNAAYEAGVEKFIYVSTDEVYGGSLDQEFDESSPKQPTNPYASSKAAAECFVQSYWERYKFPVVITRSSNVYGPHQYPEKVIPKFISLLQHNRKCCIHGSGLQRRNFLYAADVVEAFLTVLTKGEPGEIYNIGTNFEMSVVQLAKELIQLIKETNSESETESWVDYVSDRPHNDMRYPMKSEKIHSLGWKPKVPWEEGIKKTVEWYRKNFHNWKNAEKALEPFPVQPPFM
uniprot
proteins
ec_number
4.2.1.76
uniprot
proteins
function
FUNCTION: UDP-glucose 4,6-dehydratase that converts UDP-glucose into UDP-4-keto-6-deoxyglucose, and which is required for glycosaminoglycan biosynthesis and skeletal development (PubMed:40836090). UDP-4-keto-6-deoxyglucose is a mimic of the reaction intermediate of UXS1 and acts as an enzyme-rescue metabolite to promote the completion of UXS1 catalytic cycle when NAD(+) levels are low (PubMed:40836090). Under low NAD(+) conditions, UXS1 forms an inactive UDP-4-ketoxylose intermediate bound to NADH, impairing the synthesis of specific glycans that are essential for skeletal development (PubMed:40836090). UDP-4-keto-6-deoxyglucose is used by the inactive NADH-bound UXS1 to produce UDP-6-deoxyglucose and NAD(+) within the catalytic pocket of UXS1, regenerating the essential cofactor NAD(+) (By similarity). {ECO:0000250|UniProtKB:O95455, ECO:0000269|PubMed:40836090}.
uniprot
proteins
mass_dalton
40570
uniprot
proteins
mutagenesis
MUTAGEN 100; /note="A->S: Knockin mice show mild but significant reduction of the length of the skull, mandible and nasal bone. Decreased UTP-glucose 4,6-dehydratase activity."; /evidence="ECO:0000269|PubMed:40836090
uniprot
proteins
protein_family
NAD(P)-dependent epimerase/dehydratase family
uniprot
proteins
protein_family
dTDP-glucose dehydratase subfamily
uniprot
proteins
pubmed
15489334
uniprot
proteins
pubmed
16141072
uniprot
proteins
pubmed
40836090
uniprot
proteins
sequence_length
355
uniprot
proteins
subcellular_location
SUBCELLULAR LOCATION: Endoplasmic reticulum {ECO:0000250|UniProtKB:O95455}. Golgi apparatus {ECO:0000250|UniProtKB:O95455}.
uniprot
proteins