GLA MaxPab rabbit polyclonal antibody (D01)
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More Files
- More Functions
Western Blot (Cell lysate)
GLA MaxPab rabbit polyclonal antibody. Western Blot analysis of GLA expression in HeLa.
Western Blot (Transfected lysate)
Western Blot analysis of GLA expression in transfected 293T cell line (H00002717-T02) by GLA MaxPab polyclonal antibody.
Lane 1: GLA transfected lysate(48.8 KDa).
Lane 2: Non-transfected lysate.
Immunoprecipitation
Immunoprecipitation of GLA transfected lysate using anti-GLA MaxPab rabbit polyclonal antibody and Protein A Magnetic Bead, and immunoblotted with GLA purified MaxPab mouse polyclonal antibody (B01P) (H00002717-B01P).
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Specification
Product Description
Rabbit polyclonal antibody raised against a full-length human GLA protein.
Immunogen
GLA (NP_000160.1, 1 a.a. ~ 429 a.a) full-length human protein.
Sequence
MQLRNPELHLGCALALRFLALVSWDIPGARALDNGLARTPTMGWLHWERFMCNLDCQEEPDSCISEKLFMEMAELMVSEGWKDAGYEYLCIDDCWMAPQRDSEGRLQADPQRFPHGIRQLANYVHSKGLKLGIYADVGNKTCAGFPGSFGYYDIDAQTFADWGVDLLKFDGCYCDSLENLADGYKHMSLALNRTGRSIVYSCEWPLYMWPFQKPNYTEIRQYCNHWRNFADIDDSWKSIKSILDWTSFNQERIVDVAGPGGWNDPDMLVIGNFGLSWNQQVTQMALWAIMAAPLFMSNDLRHISPQAKALLQDKDVIAINQDPLGKQGYQLRQGDNFEVWERPLSGLAWAVAMINRQEIGGPRSYTIAVASLGKGVACNPACFITQLLPVKRKLGFYEWTSRLRSHINPTGTVLLQLENTMQMSLKDLL
Host
Rabbit
Reactivity
Human
Quality Control Testing
Antibody reactive against mammalian transfected lysate.
Storage Buffer
No additive
Storage Instruction
Store at -20°C or lower. Aliquot to avoid repeated freezing and thawing.
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Applications
Western Blot (Cell lysate)
GLA MaxPab rabbit polyclonal antibody. Western Blot analysis of GLA expression in HeLa.Western Blot (Transfected lysate)
Western Blot analysis of GLA expression in transfected 293T cell line (H00002717-T02) by GLA MaxPab polyclonal antibody.
Lane 1: GLA transfected lysate(48.8 KDa).
Lane 2: Non-transfected lysate.
Immunoprecipitation
Immunoprecipitation of GLA transfected lysate using anti-GLA MaxPab rabbit polyclonal antibody and Protein A Magnetic Bead, and immunoblotted with GLA purified MaxPab mouse polyclonal antibody (B01P) (H00002717-B01P). -
Gene Info — GLA
Entrez GeneID
2717GeneBank Accession#
NM_000169.1Protein Accession#
NP_000160.1Gene Name
GLA
Gene Alias
GALA
Gene Description
galactosidase, alpha
Gene Ontology
HyperlinkGene Summary
This gene encodes a homodimeric glycoprotein that hydrolyses the terminal alpha-galactosyl moieties from glycolipids and glycoproteins. This enzyme predominantly hydrolyzes ceramide trihexoside, and it can catalyze the hydrolysis of melibiose into galactose and glucose. A variety of mutations in this gene affect the synthesis, processing, and stability of this enzyme, which causes Fabry disease, a rare lysosomal storage disorder that results from a failure to catabolize alpha-D-galactosyl glycolipid moieties. [provided by RefSeq
Other Designations
OTTHUMP00000023679|agalsidase alfa|alpha-D-galactosidase A|alpha-D-galactoside galactohydrolase 1|alpha-gal A|alpha-galactosidase A|melibiase
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Interactome
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Pathway
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Disease
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Publication Reference
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Functional and biological studies of α-galactosidase A variants with uncertain significance from newborn screening in Taiwan.
Liao HC, Hsu TR, Young L, Chiang CC, Huang CK, Liu HC, Niu DM, Chen YJ.
Molecular Genetics and Metabolism 2017 Jun; [Epub].
Application：WB-Ce, Human , HEK 293 cells.
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Synergy between the pharmacological chaperone 1-deoxygalactonojirimycin and the human recombinant alpha-galactosidase A in cultured fibroblasts from patients with Fabry disease.
Porto C, Pisani A, Rosa M, Acampora E, Avolio V, Tuzzi MR, Visciano B, Gagliardo C, Materazzi S, la Marca G, Andria G, Parenti G.
Journal of Inherited Metabolic Disease 2012 May; 35(3):513.
Application：IF, WB, Human, Human fibroblasts.
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Functional and biological studies of α-galactosidase A variants with uncertain significance from newborn screening in Taiwan.
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