GAA sirna

GAA siRNA (Rat)

Cat. Num. AAA8211064

• Reactivity: Rat
• Applications: RNA Interference (RNAi)
• Purity: > 97%
• Synonyms: GAA; GAA siRNA (Rat); Lysosomal alpha-glucosidase; Acid maltase; GAA sirna

• Host: Synthetic
• Reactivity: Rat
• Specificity: GAA siRNA (Rat) is a target-specific 19-23 nt siRNA oligo duplexes designed to knock down gene expression.
• Purity/Purification: > 97%
• Form/Format: Lyophilized powder
• Sequence Length: 953

• Applicable Applications for GAA sirna: RNA Interference (RNAi)
• Quality Control: Oligonucleotide synthesis is monitored base by base through trityl analysis to ensure appropriate coupling efficiency. The oligo is subsequently purified by affinity-solid phase extraction. The annealed RNA duplex is further analyzed by mass spectrometry to verify the exact composition of the duplex. Each lot is compared to the previous lot by mass spectrometry to ensure maximum lot-to-lot consistency.
• Directions for Use: We recommends transfection with 100 nM siRNA 48 to 72 hours prior to cell lysis. Before resuspending, briefly centrifuge the tube to ensure the lyophilized siRNA is at the bottom of the tube. Resuspend the siRNA oligos to an appropriate concentration with DEPC water. For each vial, suitable for 250 transfections in 24 well plate (20 pmol for each well).
• Components: We offer pre-designed sets of 3 different target-specific siRNA oligo duplexes of rat GAA gene. Each vial contains 5 nmol of lyophilized siRNA. The duplexes can be transfected individually or pooled together to achieve knockdown of the target gene, which is most commonly assessed by qPCR or western blot. Our siRNA oligos are also chemically modified (2'-OMe) at no extra charge for increased stability and enhanced knockdown in vitro and in vivo.
• Preparation and Storage: Shipped at 4 degree C. Store at -20 degree C for one year.

Related Product Information for GAA sirna

siRNA to inhibit GAA expression using RNA interference

NCBI and Uniprot Product Information

• NCBI GI #: 40018606
• NCBI GeneID: 367562
• NCBI Accession #: NP_954549.1
• NCBI GenBank Nucleotide #: NM_199118.1
• UniProt Accession #: Q6P7A9
• Molecular Weight: 106,207 Da
• NCBI Official Full Name: lysosomal alpha-glucosidase
• NCBI Official Synonym Full Names: glucosidase, alpha, acid
• NCBI Official Symbol: Gaa
• NCBI Protein Information: lysosomal alpha-glucosidase
• UniProt Protein Name: Lysosomal alpha-glucosidase
• Protein Family: Lysosomal alpha-glucosidase
• UniProt Gene Name: Gaa
• UniProt Entry Name: LYAG_RAT

NCBI Description

human homolog catalyzes the degradation of glycogen [RGD, Feb 2006]

Uniprot Description

GAA: Essential for the degradation of glygogen to glucose in lysosomes. Defects in GAA are the cause of glycogen storage disease type 2 (GSD2); also called acid alpha-glucosidase (GAA) deficiency or acid maltase deficiency (AMD). GSD2 is a metabolic disorder with a broad clinical spectrum. The severe infantile form, or Pompe disease, presents at birth with massive accumulation of glycogen in muscle, heart and liver. Cardiomyopathy and muscular hypotonia are the cardinal features of this form whose life expectancy is less than two years. The juvenile and adult forms present as limb-girdle muscular dystrophy beginning in the lower limbs. Final outcome depends on respiratory muscle failure. Patients with the adult form can be free of clinical symptoms for most of their life but finally develop a slowly progressive myopathy. Belongs to the glycosyl hydrolase 31 family.

Protein type: Carbohydrate Metabolism - starch and sucrose; Hydrolase; EC 3.2.1.20; Carbohydrate Metabolism - galactose; Contractile

Cellular Component: membrane; lysosomal membrane; lysosome

Molecular Function: alpha-glucosidase activity; maltase activity; carbohydrate binding

Biological Process: glycogen metabolic process; heart morphogenesis; tissue development; glycogen catabolic process; striated muscle contraction; vacuolar sequestering; locomotory behavior; muscle maintenance; neuromuscular process controlling posture; lysosome organization and biogenesis; diaphragm contraction; neuromuscular process controlling balance; regulation of the force of heart contraction; cardiac muscle contraction