N-Acetylgalactosaminidase Alpha (NAGA) Antibody Pair

Product specifications

Antibody Pairs

N-Acetylgalactosaminidase Alpha (NAGA)

Mouse

Detection Antibody Biotinilated

Sandwich

Dilute the Capture Antibody 125-fold with Coating Buffer.  Dilute the Biotin-Conjugated Detection Antibody 200-fold with Detection Antibody Diluent.  Optimal dilutions/concentrations should be determined by the end user.

5 × 96 tests

10 × 96 tests

Standard: Lyophilized--Liquid (Capture Antibody and Detection Antibody)

Reconstitute the standard with Standard Diluent. The volume, and therefore standard concentration, should be determined by the end user.

ELISA

The Capture and Detection Antibody both contain 0.1% sodium azide.

Please enquire.

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

No

GALB, D22S674

Antibody Pair for NAGA

RUO

This antibody pair contains ( 5 x 96 det):    Detection:  50 µg  Capture:  200 µg  Standard:  2 µg

This product is for research use only.

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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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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