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PHKB Rabbit pAb (bs-4032R)  
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產(chǎn)品編號(hào) bs-4032R
英文名稱 PHKB Rabbit pAb
中文名稱 磷酸化酶β抗體
別    名 phosphorylase B; phosphorylase kinase B; phosphorylase kinase beta polypeptide; phosphorylase kinase beta subunit; DKFZp781E15103; FLJ41698; KPBB_HUMAN.  
Specific References  (1)     |     bs-4032R has been referenced in 1 publications.
[IF=0] Terashima et al. KIAA1199 interacts with glycogen phosphorylase kinase β-subunit (PHKB) to promote glycogen breakdown and cancer cell survival. (2014) Oncotarget. 5:7040-50  IP ;  Human.  
研究領(lǐng)域 腫瘤  細(xì)胞生物  免疫學(xué)  激酶和磷酸酶  
抗體來(lái)源 Rabbit
克隆類(lèi)型 Polyclonal
克 隆 號(hào)
交叉反應(yīng) Human (predicted: Mouse,Rat,Rabbit,Pig,Cow,Dog,Horse)
產(chǎn)品應(yīng)用 WB=1:500-2000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 124 kDa
檢測(cè)分子量
細(xì)胞定位 細(xì)胞漿 細(xì)胞膜 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human PHKB: 45-160/1093 
亞    型 IgG
純化方法 affinity purified by Protein A
緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事項(xiàng) This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產(chǎn)品介紹 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, encoded by two different genes. The beta subunit is the same in both the muscle and hepatic isoforms, encoded by this gene, which is a member of the phosphorylase b kinase regulatory subunit family. The gamma subunit also includes the skeletal muscle and hepatic isoforms, 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 9B, also known as phosphorylase kinase deficiency of liver and muscle. Alternatively spliced transcript variants encoding different isoforms have been identified in this gene. Two pseudogenes have been found on chromosomes 14 and 20, respectively.[provided by RefSeq, Feb 2010].

Function:
Phosphorylase b kinase catalyzes the phosphorylation of serine in certain substrates, including troponin I. The beta chain acts as a regulatory unit and modulates the activity of the holoenzyme in response to phosphorylation.

Subunit:
Hexadecamer of 4 heterotetramers, each composed of alpha, beta, 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).

Post-translational modifications:
Ser-701 is probably phosphorylated by PKA.
Although the final Cys may be farnesylated, the terminal tripeptide is probably not removed, and the C-terminus is not methylated.

DISEASE:
Defects in PHKB are the cause of glycogen storage disease type 9B (GSD9B) [MIM:261750]; also known as phosphorylase kinase deficiency of liver and muscle (PKD). GSD9B is a metabolic disorder characterized by hepathomegaly, only slightly elevated transaminases and plasma lipids, clinical improvement with increasing age, and remarkably no clinical muscle involvement. Biochemical observations suggest that this mild phenotype is caused by an incomplete holoenzyme that lacks the beta subunit, but that may possess residual activity.

Similarity:
Belongs to the phosphorylase b kinase regulatory chain family.

SWISS:
Q93100

Gene ID:
5257

Database links:

Entrez Gene: 5257 Human

Entrez Gene: 102093 Mouse

Entrez Gene: 361377 Rat

Omim: 172490 Human

SwissProt: Q93100 Human

SwissProt: Q7TSH2 Mouse

Unigene: 78060 Human

Unigene: 237296 Mouse

Unigene: 6488 Rat



磷酸化酶A是磷酸化酶的活性狀態(tài),而磷酸化酶B是其無(wú)活性狀態(tài)。6-磷酸葡萄糖結(jié)合磷酸化酶后,磷酸化酶由無(wú)活性變?yōu)榛罨瘧B(tài),的加長(zhǎng),之后,又回到原來(lái)無(wú)活性狀態(tài),它充當(dāng)了個(gè)橋梁作用。
產(chǎn)品圖片
Sample: K562 (Human) Cell Lysate at 30 ug Primary: Anti- PHKB (bs-4032R) at 1/1000 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 124 kD Observed band size: 124 kD
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