Rat Naga(Alpha-N-acetylgalactosaminidase) ELISA Kit

Este producto es parte de NAGA-N-Acetylgalactosaminidase Alpha
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935106861
info@markelab.com
name
Rat Naga(Alpha-N-acetylgalactosaminidase) ELISA Kit
category
ELISA Kits
provider
FineTest
reference
ER0511

Documents del producto

Instrucciones
Descargar
Data sheet

Product specifications

Category
ELISA Kits
Reactivity
rat
Detection Method
Colorimetric
Assay Data
Quantitative
Assay Type
Sandwich ELISA, Double Antibody
Test Range
0.313-20ng/ml
Size 1
96T
Sample Type
Serum,Plasma,Tissue homogenates,Other biological fluids
Availability
Shipped within 10-14 working days.
Storage
2-8 °C for 6 months
UniProt ID
Q66H12
Alias
GALB, D22S674
Background
Elisa Kits NAGA
Status
RUO

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 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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