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Invitrogen™ NAGA Polyclonal Antibody
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Catalog No. PIPA547863
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100 μg
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PIPA547863 100 μg
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Catalog No. PIPA547863 Supplier Invitrogen™ Supplier No. PA547863
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Description

Sheep Polyclonal Antibody

In direct ELISAs, less than 2% cross-reactivity with recombinant human alpha -Galactosidase A is observed. Reconstitute in sterile PBS to a final concentration of 0.2 mg/mL.

NAGA (N-acetylgalactosaminidase, alpha), also known as alpha-galactosidase B or GALB, is a 411 lysosomal protein belonging to the glycosyl hydrolase 27 family that may exist as a homodimer and plays a critical role in glycolipid breakdown. NAGA encodes alpha-N-acetylgalactosaminidase, a lysosomal enzyme, which cleaves alpha-N-acetylgalactosaminyl groups from glycoconjugates. Mapping to human chromosome 22q13. 2, NAGA defects are the cause of an autosomal recessive disorder with three phenotypes, known as Schindler disease (types I, II and III) or NAGA deficiency (types I, II and III). Characterized by neurologic manifestations that range in severity, Schindler disease type I is the most severe form, followed by type III, which may have mild-to-moderate effects. Schindler disease type II, also known as Kanzaki disease, is characterized by mild intellectual impairment and angiokeratoma corporis diffusum.
TRUSTED_SUSTAINABILITY

Specifications

Antigen NAGA
Applications Western Blot
Classification Polyclonal
Concentration 0.2 mg/mL
Conjugate Unconjugated
Formulation PBS with 5% trehalose and No Preservative
Gene NAGA
Gene Accession No. P17050
Gene Alias Acetylgalactosaminidase, alpha-N- (alpha-galactosidase B); alpha-galactosidase B; Alpha-N-acetylgalactosaminidase; D22S674; EC 3.2.1; EC 3.2.1.49; galactosidasealphaB; galactosidase-alphaB; GALB; N-acetyl galactosaminidase, alpha; N-acetylgalactosaminidase, alpha-; Naga
Gene Symbols NAGA
Host Species Sheep
Immunogen CHO-derived recombinant human alpha-N-acetylgalactosaminidase/NAGA Leu18-Gln411.
Purification Method Antigen affinity chromatography
Quantity 100 μg
Regulatory Status RUO
Primary or Secondary Primary
Gene ID (Entrez) 4668
Target Species Human
Content And Storage -20°C, Avoid Freeze/Thaw Cycles
Product Type Antibody
Form Lyophilized
Isotype IgG
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