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Proteintech
GAA Polyclonal Antibody
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产品信息
14367-1-AP
应用
建议稀释比
已发表文章
产品规格
种属反应
Human,
Mouse,
Rat
已发表种属
Zebrafish
宿主/亚型
Rabbit
/ IgG
分类
Polyclonal
类型
Antibody
抗原
GAA Fusion Protein Ag5690 (601-952 aa encoded by BC040431)
偶联物
形式
Liquid
浓度
0.16 mg/mL
纯化类型
Antigen 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
运输条件
Wet ice (domestic); Dry ice (international)
产品详细信息
Immunogen sequence: STFAGHGRY AGHWTGDVWS SWEQLASSVP EILQFNLLGV PLVGADVCGF LGNTSEELCV RWTQLGAFYP FMRNHNSLLS LPQEPYSFSE PAQQAMRKAL TLRYALLPHL YTLFHQAHVA GETVARPLFL EFPKDSSTWT VDHQLLWGEA LLITPVLQAG KAEVTGYFPL GTWYDLQTVP VEALGSLPPP PAAPREPAIH SEGQWVTLPA PLDTINVHLR AGYIIPLQGP GLTTTESRQQ PMALAVALTK GGEARGELFW DDGESLEVLE RGAYTQVIFL ARNNTIVNEL VRVTSEGAGL QLQKVTVLGV ATAPQQVLSN GVPVSNFTYS PDTKVLDICV SLLMGEQFLV SWC (601-952 aa encoded by BC040431)
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。
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
