Galactose-1-phosphate uridylyltransferase

Available protein structures:
Pfam	structures / ECOD
PDB	RCSB PDB; PDBe; PDBj
PDBsum	structure summary

Galactose-1-phosphate uridyl transferase, N-terminal domain
Galactose-1-phosphate uridyl transferase, N-terminal domain
Identifiers
Symbol	GalP_UDP_transf
Pfam	PF01087
Pfam clan	CL0265
PROSITE	PDOC00108
SCOP2	1hxp / SCOPe / SUPFAM
Pfam
Available protein structures:
Pfam	structures / ECOD
PDB	RCSB PDB; PDBe; PDBj
PDBsum	structure summary

GALT
Available structures
PDB	Ortholog search: PDBe RCSB
List of PDB id codes
	5IN3
Identifiers
Aliases	GALT, entrez:2592, galactose-1-phosphate uridylyltransferase
External IDs	OMIM: 606999 MGI: 95638 HomoloGene: 126 GeneCards: GALT
Gene location (Human)
Chr.	Chromosome 9 (human)
End	34,651,035 bp
Gene location (Mouse)
Chr.	Chromosome 4 (mouse)
End	41,758,695 bp
RNA expression pattern
	Top expressed in
	right lobe of liver; ; right adrenal gland; ; right lobe of thyroid gland; ; anterior pituitary; ; left lobe of thyroid gland; ; right coronary artery; ; tibial nerve; ; right uterine tube; ; left uterine tube; ; spleen;
	Top expressed in
	superior frontal gyrus; ; cerebellum; ; cerebellar cortex; ; renal cortex; ; proximal tubule; ; heart; ; adrenal gland; ; muscle tissue; ; kidney; ; olfactory bulb;
	More reference expression data
	n/a
Gene ontology
Molecular function	transferase activity; nucleotidyltransferase activity; protein binding; metal ion binding; UDP-glucose:hexose-1-phosphate uridylyltransferase activity; zinc ion binding;
Cellular component	cytosol; Golgi apparatus; cytoplasm;
Biological process	UDP-glucose catabolic process; galactose catabolic process; galactose catabolic process via UDP-galactose; UDP-glucose metabolic process; galactose metabolic process; carbohydrate metabolic process;
	Sources:Amigo / QuickGO
Orthologs
	2592
	14430
	ENSG00000213930
	ENSMUSG00000036073
	P07902
	Q03249
	NM_001258332; NM_000155; NM_147131; NM_147132
	NM_016658; NM_001302511
	NP_000146; NP_001245261
NP_001289440; NP_057867; NP_001356064; NP_001356069; NP_001356070;
	NP_001356071; NP_001356072; NP_001356073; NP_001356076; NP_001382559
	Wikidata
View/Edit Human	View/Edit Mouse

Galactose-1-phosphate uridyltransferase (or GALT, G1PUT) is an enzyme (EC 2.7.7.12) responsible for converting ingested galactose to glucose.

Galactose-1-phosphate uridyl transferase, C-terminal domain

structure of nucleotidyltransferase complexed with udp-galactose

Identifiers

Symbol

GalP_UDP_tr_C

Pfam

Pfam clan

PROSITE

SCOP2

1hxp / SCOPe / SUPFAM

Available protein structures:
Pfam	structures / ECOD
PDB	RCSB PDB; PDBe; PDBj
PDBsum	structure summary

Galactose-1-phosphate uridyltransferase (GALT) catalyzes the second step of the Leloir pathway of galactose metabolism, namely:

UDP-glucose + galactose 1-phosphate

\rightleftharpoons

glucose 1-phosphate + UDP-galactose

The expression of GALT is controlled by the actions of the FOXO3 gene. The absence of this enzyme results in classic galactosemia in humans and can be fatal in the newborn period if lactose is not removed from the diet. The pathophysiology of galactosemia has not been clearly defined.

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