GAA polyclonal (N-term), anti-human
€0.00
In stock
SKU
AC-AP12544a
Catalog Number: AC-AP12544a
Size: 80 µl, 400 µl
Isotype: Rabbit IgG
Clone Name: RB18979
Applications: IHC-P-Leica, WB, IHC
Datasheet
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Size: 80 µl, 400 µl
Isotype: Rabbit IgG
Clone Name: RB18979
Applications: IHC-P-Leica, WB, IHC
Datasheet
Request Information
Background:
This gene encodes acid alpha-glucosidase, which is essential for the degradation of glycogen to glucose in lysosomes. Different forms of acid alpha-glucosidase are obtained by proteolytic processing. Defects in this gene are the cause of glycogen storage disease II, also known as Pompe's disease, which is an autosomal recessive disorder with a broad clinical spectrum. Three transcript variants encoding the same protein have been found for this gene.
Other Names:
Lysosomal alpha-glucosidase, Acid maltase, Aglucosidase alfa, 76 kDa lysosomal alpha-glucosidase, 70 kDa lysosomal alpha-glucosidase, GAA
Target/Specificity:
This GAA antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 174-203 amino acids from the N-terminal region of human GAA.
Antigen Region:
174-203
Antigen Type: Synthetic Peptide
Gene Name: GAA
Gene ID: 2548
Primary Accession: P10253
NCBI Accession: NP_000143.2;NP_001073271.1;NP_001073272.1
Other Accession: NP_000143.2;NP_001073271.1
Format: Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification.
This gene encodes acid alpha-glucosidase, which is essential for the degradation of glycogen to glucose in lysosomes. Different forms of acid alpha-glucosidase are obtained by proteolytic processing. Defects in this gene are the cause of glycogen storage disease II, also known as Pompe's disease, which is an autosomal recessive disorder with a broad clinical spectrum. Three transcript variants encoding the same protein have been found for this gene.
Other Names:
Lysosomal alpha-glucosidase, Acid maltase, Aglucosidase alfa, 76 kDa lysosomal alpha-glucosidase, 70 kDa lysosomal alpha-glucosidase, GAA
Target/Specificity:
This GAA antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 174-203 amino acids from the N-terminal region of human GAA.
Antigen Region:
174-203
Antigen Type: Synthetic Peptide
Gene Name: GAA
Gene ID: 2548
Primary Accession: P10253
NCBI Accession: NP_000143.2;NP_001073271.1;NP_001073272.1
Other Accession: NP_000143.2;NP_001073271.1
Format: Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification.
| Is Featured? | No |
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