N-Acetylgalactosaminidase Alpha (NAGA) Antibody

Product specifications

Primary Antibodies

N-Acetylgalactosaminidase Alpha (NAGA)

Rabbit

Human

WB: 0.01-2 µg/ml, IHC: 5-20 µg/ml, IF/ICC: 5-20 µg/ml. Optimal dilutions/concentrations should be determined by the end user.

Polyclonal

Unconjugated

Purified by antigen-specific affinity chromatography, followed by Protein A affinity chromatography.

100 µl

200 µl

1 ml

Liquid

WB, IHC, IF/ICC

0.01 M PBS, pH 7.4, containing 0.05% Proclin-300, 50% glycerol.

Shipped within 5-7 working days.

Aliquot and store at -20°C. Avoid repeated freeze/thaw cycles.

No

P17050

GALB, D22S674

Antibody anti-NAGA

RUO

Concentration: 1 mg/ml - 

Descripción

N-Acetylgalactosaminidase Alpha (NAGA) is an enzyme involved in the breakdown of complex carbohydrates. catalyzes the hydrolysis of terminal alpha-N-acetylgalactosaminyl residues in glycoproteins and glycolipids (lysosomes), Deficiency in NAGA enzyme activity leads to a rare genetic disorder known as Schindler disease, which is characterized by the accumulation of certain glycoproteins and glycolipids in lysosomes. Schindler disease can manifest in different forms, including Schindler disease type I and type II, depending on the severity of the enzyme deficiency. Symptoms of Schindler disease can vary widely but may include developmental delay, intellectual disability, seizures, skeletal abnormalities, and other neurological and systemic manifestations. The severity and progression of the disease can also vary, ranging from mild to severe.

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anti- NAGA antibody

NAGA belongs to the glycosyl hydrolase 27 family. It removes terminal alpha-N-acetylgalactosamine residues from glycolipids and glycopeptides. It is required for the breakdown of glycolipids. Biosynthetic studies performed with cultured fibroblasts indicated that the human enzyme was synthesized as a 65kDa glycosylated precursor which was processed to a mature 48-kDa lysosomal form; both the precursor and mature forms had high mannose type oligosaccharide chains, but only the precursor’s mannose residues were phosphorylated. 90-117kd is a homodimer of NAGA.

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