TEL: +86 571 56623320 EMAIL: [email protected]
Product Name Anti-GALE/HRP Chinese Name 辣根过氧化物酶标记的半乳糖瓦尔登转化酶抗体 Alias FLJ95174; FLJ97302; Galactose 4 epimerase UDP; Galactowaldenase; galE; GALE_HUMAN; OTTHUMP00000002991; OTTHUMP00000002994; OTTHUMP00000037931; OTTHUMP00000044857; SDR1E1; short chain dehydrogenase/reductase family 1E member 1; UDP galactose 4 epimerase; UDP glucose 4 epimerase; UDP-galactose 4-epimerase; UDP-glucose 4-epimerase. Research Area Tumour Cell biology The new supersedes the old Immunogen Species Rabbit Clonality Polyclonal React Species (predicted: Human, Mouse, Rat, Pig, Horse, ) Applications WB=1:500-2000 ELISA=1:100-1000 IHC-P=1:50-200 IHC-F=1:50-200 ICC=1:50-200
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.Molecular weight 38kDa Form Lyophilized or Liquid Concentration 1mg/ml immunogen KLH conjugated synthetic peptide derived from human GALE/Galactowaldenase Lsotype IgG Purification affinity purified by Protein A Storage Buffer 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. Storage Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C. Product Detail background:
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. [provided by RefSeq, Jul 2008]
Function:
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.
Subunit:
Homodimer.
DISEASE:
Defects in GALE are the cause of epimerase-deficiency galactosemia (EDG) [MIM:230350]; also known as galactosemia type 3. Clinical features include early-onset cataracts, liver damage, deafness and mental retardation. There are two clinically distinct forms of EDG. (1) A benign, or 'peripheral' form with no detectable GALE activity in red blood cells and characterized by mild symptoms. Some patients may suffer no symptoms beyond raised levels of galactose-1-phosphate in the blood. (2) A much rarer 'generalized' form with undetectable levels of GALE activity in all tissues and resulting in severe features such as restricted growth and mental development.
Similarity:
Belongs to the sugar epimerase family.
Database links:Entrez Gene: 2582 Human
Entrez Gene: 74246 Mouse
Omim: 606953 Human
SwissProt: Q14376 Human
SwissProt: Q8R059 Mouse
Unigene: 632380 Human
Unigene: 247946 Mouse
Unigene: 29908 Rat
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
Scan Wechat Qrcode