Lysosomal alpha-glucosidase (Gaa) Recombinant Protein | Gaa recombinant protein

Recombinant Mouse Lysosomal alpha-glucosidase (Gaa) , partial

Cat. Num. AAA967253

• Gene Names: Gaa; E430018M07Rik
• Purity: Greater or equal to 85% purity as determined by SDS-PAGE.
• Synonyms: Lysosomal alpha-glucosidase (Gaa); Recombinant Mouse Lysosomal alpha-glucosidase (Gaa); partial; Gaa recombinant protein

• Host: E Coli or Yeast or Baculovirus or Mammalian Cell
• Purity/Purification: Greater or equal to 85% purity as determined by SDS-PAGE.
• Form/Format: Lyophilized or liquid (Format to be determined during the manufacturing process)
• Sequence Length: 953

• Species: Mouse
• Preparation and Storage: Store at -20 degrees C. For long-term storage, store at -20 degrees C or -80 degrees C. Store working aliquots at 4 degrees C for up to one week. Repeated freezing and thawing is not recommended.

Related Product Information for Gaa recombinant protein

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.

NCBI and Uniprot Product Information

• NCBI GI #: 226693369
• NCBI GeneID: 14387
• NCBI Accession #: NP_001152796.1
• NCBI GenBank Nucleotide #: NM_001159324.2
• UniProt Accession #: P70699; Q3UJB2; Q8BGI6; Q91Z45
• Molecular Weight: 106,248 Da
• NCBI Official Full Name: lysosomal alpha-glucosidase preproprotein
• NCBI Official Synonym Full Names: glucosidase, alpha, acid
• NCBI Official Symbol: Gaa
• NCBI Official Synonym Symbols: E430018M07Rik
• NCBI Protein Information: lysosomal alpha-glucosidase
• UniProt Protein Name: Lysosomal alpha-glucosidase
• Protein Family: Lysosomal alpha-glucosidase
• UniProt Gene Name: Gaa

NCBI Description

This gene encodes a lysosomal acid glucosidase that is involved in the degradation of glycogen. The encoded preproprotein undergoes proteolytic processing to generate a mature enzyme that cleaves alpha-1-4 and alpha-1-6 glycosidic bonds of glycogen, maltose and intermediate oligosaccharides within the lysosome. Mice lacking the encoded protein exhibit symptoms similar to human Pompe syndrome such as accumulation of glycogen in cardiac and skeletal muscle lysosomes resulting in reduced mobility and strength. Alternative splicing of this gene results in multiple transcript variants. [provided by RefSeq, Nov 2015]

Uniprot Description

Essential for the degradation of glycogen in lysosomes. Has highest activity on alpha-1,4-linked glycosidic linkages, but can also hydrolyze alpha-1,6-linked glucans.