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产品信息
PA5-37015
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
Synthetic peptide corresponding to amino acids 699-744 of human PHKB
偶联物
形式
Liquid
浓度
1 mg/mL
纯化类型
Antigen affinity chromatography
保存液
PBS, pH 7.2, with 50% glycerol
内含物
0.02% sodium azide
保存条件
Store at 4°C short term. For long term storage, store at -20°C, avoiding freeze/thaw cycles.
运输条件
Wet ice
RRID
AB_2553867
产品详细信息
This antibody detects endogenous protein at a molecular weight of 125 kDa.
Purity is >95% by SDS-PAGE.
靶标信息
Phosphorylase kinase is a hexadecameric enzyme that is comprised of four copies of four subunits that are encoded by four separate genes: PHKA, PHKB, PHKG, and PHKD. This serine/threonine specific kinase converts glycogen phosphorylase b to glycogen phosphorylase a, resulting in the release of glucose-1-phophate from glycogen. PHKB (Phosphorylase b kinase regulatory subunit beta) is a 1093 amino acid subunit of phosphorylase kinase that, along with PHKA, has regulatory functions controlled by phosphorylation. Defects in the gene encoding PHKB are the cause of glycogen storage disease type 9B, which is also known as phosphorylase kinase deficiency of liver and muscle. This disease is characterized by a mild phenotype of hepatomegaly with only slightly elevated transaminase and plasma lipids, no clinical muscle involvement, and generally is correlated with a gradual improvement with increasing age. There are four isoforms of PHKB that are produced as a result of alternative splicing events.
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: Phosphorylase B; Phosphorylase b kinase regulatory subunit beta; phosphorylase kinase; phosphorylase kinase beta subunit; phosphorylase kinase beta-subunit; Phosphorylase kinase subunit beta; phosphorylase kinase, beta
基因别名: AI463271; PHKB
UniProt ID: (Human) Q93100, (Mouse) Q7TSH2
Entrez Gene ID: (Human) 5257, (Mouse) 102093, (Rat) 361377
