磷酸激酶α1抗体
规格：1mg/1ml
英文名： PHKA1
别名： 5330411D17; 9830108K24Rik; kinase PHKA1; KPB1; KPB1_HUMAN; MGC132604; Pcyt1b; PHKA; PHKA1; Phosphorylase b kinase regulatory subunit alpha; Phosphorylase b kinase regulatory subunit alpha, skeletal mu
分子量： 137kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：Polyclonal
亚型：IgG
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human PHKA1
交叉反应：Human, Mouse, Rat, Dog, Cow, Horse, Rabbit, Sheep,
细胞定位：
磷酸激酶α1抗体产品介绍：background: Phosphorylase kinase is a polymer of 16 subunits, four each of alpha, beta, gamma and delta. The alpha subunit includes the skeletal muscle and hepatic isoforms, and the skeletal muscle isoform is encoded by this gene. The beta subunit is the same in both the muscle and hepatic isoforms, and encoded by one gene. The gamma subunit also includes the skeletal muscle and hepatic isoforms, which are encoded by two different genes. The delta subunit is a calmodulin and can be encoded by three different genes. The gamma subunits contain the active site of the enzyme, whereas the alpha and beta subunits have regulatory functions controlled by phosphorylation. The delta subunit mediates the dependence of the enzyme on calcium concentration. Mutations in this gene cause glycogen storage disease type 9D, also known as X-linked muscle glycogenosis. Alternatively spliced transcript variants encoding different isoforms have been identified in this gene. A pseudogene has been found on chromosome 1. Function: Phosphorylase b kinase catalyzes the phosphorylation of serine in certain substrates, including troponin I. The alpha chain may bind calmodulin. Subunit: Hexadecamer of 4 heterotetramers, each composed of alpha, beta,磷酸激酶α1抗体 gamma, and delta subunits. Alpha (PHKA1 or PHKA2) and beta (PHKB) are regulatory subunits, gamma (PHKG1 or PHKG2) is the catalytic subunit, and delta is calmodulin. Subcellular Location: Cell membrane; Lipid-anchor; Cytoplasmic side (Potential). Tissue Specificity: Muscle specific. Isoform 1 is predominant in vastus lateralis muscle. Isoform 2 predominates slightly in heart, and it predominates clearly in the other tissues tested. Post-translational modifications: Although the final Cys may be farnesylated, the terminal tripeptide is probably not removed, and the C-terminus is not methylated (By similarity). DISEASE: Glycogen storage disease 9D (GSD9D) [MIM:300559]: A metabolic disorder characterized by slowly 磷酸激酶α1抗体progressive, predominantly distal muscle weakness and atrophy. Clinical features include exercise intolerance with early fatigability, pain, cramps and occasionally myoglobinuria. Note=The disease is caused by mutations affecting the gene represented in this entry. Similarity: Belongs to the phosphorylase b kinase regulatory chain family. Database links: UniProtKB/Swiss-Prot: P46020.2 Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
磷酸激酶α1抗体产品应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
研究领域：肿瘤 细胞生物 **学 信号转导 激酶和磷酸酶
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid