GALE Antibody - N-terminal region : Biotin (ARP54393_P050-Biotin)
Product Specifications
Gene Name
UDP-galactose-4-epimerase
Gene Aliases
SDR1E1
Gene ID
2582
Swiss Prot
Q14376
Accession Number
NP_001008217
Host
Rabbit
Reactivity
Human, Mouse, Rat, Cow, Dog, Guinea Pig, Pig, Rabbit
Immunogen
The immunogen is a synthetic peptide directed towards the N terminal region of human GALE
Target
GALE is an UDP-galactose-4-epimerase which catalyzes two distinct but analogous reactions: the epimerization of UDP-glucose to UDP-galactose, and the epimerization of UDP-N-acetylglucosamine to UDP-N-acetylgalactosamine. The bifunctional nature of the enzyme has the important metabolic consequence that mutant cells (or individuals) are dependent not only on exogenous galactose, but also on exogenous N-acetylgalactosamine as a necessary precursor for the synthesis of glycoproteins and glycolipids. Mutations in this gene result in epimerase-deficiency galactosemia, also referred to as galactosemia type 3, a disease characterized by liver damage, early-onset cataracts, deafness and mental retardation, with symptoms ranging from mild ('peripheral' form) to severe ('generalized' form) . This gene encodes UDP-galactose-4-epimerase which catalyzes two distinct but analogous reactions: the epimerization of UDP-glucose to UDP-galactose, and the epimerization of UDP-N-acetylglucosamine to UDP-N-acetylgalactosamine. The bifunctional nature of the enzyme has the important metabolic consequence that mutant cells (or individuals) are dependent not only on exogenous galactose, but also on exogenous N-acetylgalactosamine as a necessary precursor for the synthesis of glycoproteins and glycolipids. Mutations in this gene result in epimerase-deficiency galactosemia, also referred to as galactosemia type 3, a disease characterized by liver damage, early-onset cataracts, deafness and mental retardation, with symptoms ranging from mild ('peripheral' form) to severe ('generalized' form) . Multiple alternatively spliced transcripts encoding the same protein have been identified.
Partner Proteins
GALE; SUMO2; UBC; BAG3; FN1; APP
Clonality
Polyclonal
Conjugation
Biotin
Type
Polyclonal Antibody
Applications
WB
Purification
Affinity Purified
Concentration
0.5 mg/ml
Homology
Cow: 93%; Dog: 86%; Guinea Pig: 86%; Human: 100%; Mouse: 91%; Pig: 91%; Rabbit: 92%; Rat: 91%
Format
Liquid. Purified antibody supplied in 1x PBS buffer.
Reconstitution
All conjugated antibodies should be stored in light-protected vials or covered with a light protecting material (i.e. aluminum foil) . Conjugated antibodies are stable for at least 12 months at 4C. If longer storage is desired (24 months), conjugates may be diluted with up to 50% glycerol and stored at -20C to -80C. Freezing and thawing conjugated antibodies will compromise enzyme activity as well as antibody binding.
Molecular Weight
38kDa
References & Citations
Chhay, J.S., (2008) J. Inherit. Metab. Dis. 31 (1), 108-116
Shipping Conditions
Wet Ice
Protein Length
348
NCBI Gene Symbol
GALE
NCBI GB Accession Number
GALE
Host or Source
Rabbit
Protein Name
UDP-glucose 4-epimerase
Nucleotide Accession Number
NM_001008216
Peptide Sequence
AEKVLVTGGAGYIGSHTVLELLEAGYLPVVIDNFHNAFRGGGSLPESLRR
Frequently Asked Questions
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