Product Name	Galactosidase alpha
Chinese Name	α-半乳糖苷酶抗体
Alias	Galactosidase alpha; Alpha D galactosidase A; Alpha D galactoside galactohydrolase; Melibiase; Alpha galactosidase A; GALA; GLA; GLA protein; AGAL_HUMAN.
Research Area	Cardiovascular  Cell biology  immunology  Neurobiology
Immunogen Species	Rabbit
Clonality	Polyclonal
React Species	Human, Rat,  (predicted: Mouse, Dog, Pig, Cow, Rabbit, )
Applications	ELISA=1:5000-10000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500 （Paraffin sections need antigen repair） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
Theoretical molecular weight	45kDa
Cellular localization	cytoplasmic
Form	Liquid
Concentration	1mg/ml
immunogen	KLH conjugated synthetic peptide derived from human Galactosidase alpha: 101-200/429
Lsotype	IgG
Purification	affinity purified by Protein A
Buffer Solution	0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
Storage	Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
Attention	This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed	PubMed
Product Detail	Galactosidase alpha is involved in the hydrolysis of terminal, non reducing alpha D galactose residues in alpha D galactosides, including galactose oligosaccharides, galactomannans and galactohydrolase. Defects in GLA are the cause of Fabry's disease (FD). FD is a rare X-linked sphingolipidosis disease where glycolipid accumulates in many tissues. Clinical recognition in males results from characteristic skin lesions (angiokeratomas) over the lower trunk. Patients may show ocular deposits, febrile episodes, and burning pain in the extremities. Death results from renal failure, cardiac or cerebral complications of hypertension or other vascular disease. Heterozygous females may exhibit the disorder in an attenuated form, they are more likely to show corneal opacities. Subunit: Homodimer. Subcellular Location: Lysosome. DISEASE: Defects in GLA are the cause of Fabry disease (FD) [MIM:301500]. FD is a rare X-linked sphingolipidosis disease where glycolipid accumulates in many tissues. The disease consists of an inborn error of glycosphingolipid catabolism. FD patients show systemic accumulation of globotriaoslyceramide (Gb3) and related glycosphingolipids in the plasma and cellular lysosomes throughout the body. Clinical recognition in males results from characteristic skin lesions (angiokeratomas) over the lower trunk. Patients may show ocular deposits, febrile episodes, and burning pain in the extremities. Death results from renal failure, cardiac or cerebral complications of hypertension or other vascular disease. Heterozygous females may exhibit the disorder in an attenuated form, they are more likely to show corneal opacities. Similarity: Belongs to the glycosyl hydrolase 27 family. SWISS: P06280 Gene ID: 2717 Database links: Entrez Gene: 2717 Human Entrez Gene: 11605 Mouse Entrez Gene: 363494 Rat Omim: 300644 Human SwissProt: P06280 Human SwissProt: P51569 Mouse Unigene: 69089 Human Unigene: 1114 Mouse
Product Picture	Paraformaldehyde-fixed, paraffin embedded (rat brain); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (Galactosidase alpha) Polyclonal Antibody, Unconjugated (SL7593R) at 1:200 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining. Tissue/cell: human rectal carcinoma; 4% Paraformaldehyde-fixed and paraffin-embedded; Antigen retrieval: citrate buffer ( 0.01M, pH 6.0 ), Boiling bathing for 15min; Block endogenous peroxidase by 3% Hydrogen peroxide for 30min; Blocking buffer (normal goat serum,C-0005) at 37℃ for 20 min; Incubation: Anti-Galactosidase alpha Polyclonal Antibody, Unconjugated(SL7593R) 1:200, overnight at 4°C, followed by conjugation to the secondary antibody(SP-0023) and DAB(C-0010) staining

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