NAGA antibody

Este producto es parte de NAGA-N-Acetylgalactosaminidase Alpha
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935106861
info@markelab.com
name
NAGA antibody
category
Primary Antibodies
provider
FineTest
reference
FNab05537
tested applications
ELISA, WB, IHC, IF

Description

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.

Documents del producto

Instrucciones
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Data sheet
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Product specifications

Category
Primary Antibodies
Immunogen Target
N-acetylgalactosaminidase, alpha- (NAGA)
Host
Rabbit
Reactivity
Human, Mouse, Rat
Recommended Dilution
WB: 1:500-1:2000; IHC: 1:20-1:200; IF: 1:20-1:200
Clonality
polyclonal
Conjugation
Unconjugated
Isotype
IgG
Observed MW
48 kDa, 60-70 kDa
Purity
≥95% as determined by SDS-PAGE
Purification
Immunogen affinity purified
Size 1
100µg
Form
liquid
Tested Applications
ELISA, WB, IHC, IF
Storage
PBS with 0.02% sodium azide and 50% glycerol pH 7.3, -20℃ for 12 months(Avoid repeated freeze / thaw cycles.)
UniProt ID
P17050
Gene ID
4668
Alias
GALB, D22S674
Background
Antibody anti-NAGA
Status
RUO
Note
Mol. Weight 48 kDa, 60-70 kDa

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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FNab05537

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.

Ver Producto