PHKA2 Antibody
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中文名稱：PHKA2兔多克隆抗體
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貨號：CSB-PA572789
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規格：￥1100
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圖片：
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The image on the left is immunohistochemistry of paraffin-embedded Human prostate cancer tissue using CSB-PA572789(PHKA2 Antibody) at dilution 1/30, on the right is treated with synthetic peptide. (Original magnification: ×200) -
The image on the left is immunohistochemistry of paraffin-embedded Human brain tissue using CSB-PA572789(PHKA2 Antibody) at dilution 1/30, on the right is treated with synthetic peptide. (Original magnification: ×200)
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其他：
產品詳情
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Uniprot No.：
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基因名：
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別名：PHKA2 antibody; PHKLA antibody; PYKPhosphorylase b kinase regulatory subunit alpha antibody; liver isoform antibody; Phosphorylase kinase alpha L subunit antibody
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宿主：Rabbit
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反應種屬：Human
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免疫原：Synthetic peptide of Human PHKA2
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免疫原種屬：Homo sapiens (Human)
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標記方式：Non-conjugated
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抗體亞型：IgG
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純化方式：Antigen affinity purification
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濃度：It differs from different batches. Please contact us to confirm it.
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保存緩沖液：-20°C, pH7.4 PBS, 0.05% NaN3, 40% Glycerol
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產品提供形式：Liquid
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應用范圍：ELISA,IHC
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推薦稀釋比：
Application Recommended Dilution ELISA 1:1000-1:2000 IHC 1:25-1:100 -
Protocols：
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儲存條件：Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
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貨期：Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
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用途：For Research Use Only. Not for use in diagnostic or therapeutic procedures.
產品評價
相關產品
靶點詳情
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功能：Phosphorylase b kinase catalyzes the phosphorylation of serine in certain substrates, including troponin I. The alpha chain may bind calmodulin.
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基因功能參考文獻：
- Clinical data and analyzed the PHKA2 gene of 17 Chinese male patients suspected of having Glycogen storage disease (GSD) type IXa; study detected 14 mutations in 17 patients, including 8 novel mutations; exons 2 and 4 were hot spots. PMID: 28627441
- In this study we summarized the PHKA2 mutation spectrum in Korean glycogen storage disease type IX patients and found that the most common mutation type was gross deletion. PMID: 27103379
- The present case also represents the first known reported case of liver PhK deficiency with an PHKA2 mutation and liver cirrhosis. PMID: 21857251
- We found that the missense mutation p.Pro1205Leu in the PHKA2 gene is a common cause of hepatic phosphorylase-kinase deficiency in Dutch patients, suggesting a founder-effect PMID: 21911307
- two novel mutations found in two GSD type IX patients with different residual enzyme activities PMID: 21131218
- Gene analysis was confirmed to represent a useful procedure for diagnosing x-linked liver glycogenosis, for which liver biopsy had previously been required to detect hepatic phosphorylase kinase deficiency PMID: 12862311
- Alu-mediated, large deletion-spanning introns 19-26 in PHKA2 is associated with X-linked liver glycogenosis PMID: 17581768
- Results show that phosphorylase kinase (PhK) alpha subunit missense mutations or small in-frame deletions/insertions may have a direct impact on the PhK alpha functions. PMID: 18950708
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相關疾病：Glycogen storage disease 9A (GSD9A)
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亞細胞定位：Cell membrane; Lipid-anchor; Cytoplasmic side.
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蛋白家族：Phosphorylase b kinase regulatory chain family
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組織特異性：Predominantly expressed in liver and other non-muscle tissues.
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數據庫鏈接：
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