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GAA Antibody (PA5-96756) in WB
Western blot analysis of GAA in extracts of various cell lines. Samples were incubated with GAA Polyclonal antibody (Product # PA5-96756) using a dilution of 1:1,000, followed by HRP Goat Anti-Rabbit IgG (H+L) at a dilution of 1:10,000. Lysates/proteins: 25 µg per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit. Exposure time: 60s.
产品信息
PA5-96756
产品规格
种属反应
Human,
Mouse,
Rat
宿主/亚型
Rabbit
/ IgG
分类
Polyclonal
类型
Antibody
抗原
A synthetic peptide corresponding to a sequence within amino acids 350-450 of human GAA (NP_0001432)
偶联物
形式
Liquid
浓度
2.91 mg/mL
纯化类型
Affinity Chromatography
保存液
PBS, pH 7.3, with 50% glycerol
内含物
0.02% sodium azide
保存条件
-20° C, Avoid Freeze/Thaw Cycles
运输条件
Wet ice
RRID
AB_2808558
产品详细信息
Immunogen sequence: VVQQYLDVVG YPFMPPYWGL GFHLCRWGYS STAITRQVVE NMTRAHFPLD VQWNDLDYMD SRRDFTFNKD GFRDFPAMVQ ELHQGGRRYM MIVDPAISSS G; Positive Samples: LO2, HT-29, 293T, A-549, DU145, Mouse liver, Mouse heart, Mouse thymus, Rat liver; Cellular Location: Lysosome, Lysosome membrane
靶标信息
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.
仅用于科研。不用于诊断过程。未经明确授权不得转售。
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生物信息学
蛋白别名: acid (Pompe disease, glycogen storage disease type II); acid alpha-glucosidase; Acid maltase; Aglucosidase alfa; glucosidase, alpha; acid (Pompe disease, glycogen storage disease type II); Lysosomal alpha-glucosidase
基因别名: E430018M07Rik; GAA; LYAG
UniProt ID: (Human) P10253, (Mouse) P70699, (Rat) Q6P7A9
Entrez Gene ID: (Human) 2548, (Mouse) 14387, (Rat) 367562
maltose metabolic process
regulation of the force of heart contraction
diaphragm contraction
heart morphogenesis
glycogen catabolic process
sucrose metabolic process
glucose metabolic process
lysosome organization
locomotory behavior
tissue development
vacuolar sequestering
muscle cell cellular homeostasis
neuromuscular process controlling posture
neuromuscular process controlling balance
cardiac muscle contraction
carbohydrate metabolic process
glycogen metabolic process
striated muscle contraction
metabolic process
