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KPB2 rabbit pAb

商品貨號： PLA020405

適應(yīng) 性： 人,小鼠

￥600元

規(guī)格：

40μL 100μL 200μL

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MSDS

說明書

商品描述

基因名稱： PHKA2 PHKLA PYK
蛋白名稱： KPB2
Human_gene_id： 5256
Human_gene_link： http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=5256
Human_swiss_prot_no： P46019
Human_swiss_link： https://www.uniprot.org/uniprotkb/P46019/entry
Mouse_gene_id： 110094
Mouse_gene_link： https://www.uniprot.org/uniprot/110094
Mouse_swiss_prot_no： Q8BWJ3
Mouse_swiss_link： https://www.uniprot.org/uniprotkb/Q8BWJ3
特異性： This antibody detects endogenous levels of KPB2 at Human/Mouse
組成： Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
來源： Polyclonal, Rabbit,IgG
稀釋： WB 1:500-2000;IHC-p 1:50-300
純化工藝： The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
濃度： 1 mg/ml
儲存： -15°C to -25°C/1 year(Do not lower than -25°C)
說明書： 1
Msds： MSDS_Antibody.pdf
分子量： 136kD
功能： disease:Defects in PHKA2 are the cause of glycogen storage disease type 9A (GSD9A) [MIM:306000]; also known as X-linked liver glycogenosis (XLG). GSD9A is a metabolic disorder resulting in a mild glycogenosis with clinical symptoms that include hepatomegaly, growth retardation, muscle weakness, elevation of glutamate-pyruvate transaminase and glutamate-oxaloacetate transaminase, hypercholesterolemia, hypertriglyceridemia, and fasting hyperketosis. Two subtypes are known: type 1 or classic type, and type 2 or variant type. The variant type is characterized mainly by enlarged liver and growth retardation; patients do not show in vitro enzymatic deficiency of phosphorylase kinase. Unlike other glycogenosis diseases, GSD9A is generally a benign condition. Patients improve with age and are often asymptomatic as adults. Accurate diagnosis is therefore also of prognostic interest.,enzyme regulation:By phosphorylation of various serine residues and by calcium.,function:Phosphorylase b kinase catalyzes the phosphorylation of serine in certain substrates, including troponin I. The alpha chain may bind calmodulin.,pathway:Glycan biosynthesis; glycogen metabolism.,similarity:Belongs to the phosphorylase b kinase regulatory chain family.,subunit:Polymer of 16 chains, four each of alpha, beta, gamma, and delta. Alpha and beta are regulatory chains, gamma is the catalytic chain, and delta is calmodulin.,tissue specificity:Predominantly expressed in liver and other non-muscle tissues.,
相關(guān)產(chǎn)品： RS0001,RS0002,YM3028,YM3029
細(xì)胞定位： Cell membrane ; Lipid-anchor ; Cytoplasmic side .
組織表達(dá)： Predominantly expressed in liver and other non-muscle tissues.
科研貨號： PLA020405

KPB2 rabbit pAb

Catalog No PLA020405

Product information

基因名稱： PHKA2 PHKLA PYK
蛋白名稱： KPB2
Human_gene_id： 5256
Human_gene_link： http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=5256
Human_swiss_prot_no： P46019
Human_swiss_link： https://www.uniprot.org/uniprotkb/P46019/entry
Mouse_gene_id： 110094
Mouse_gene_link： https://www.uniprot.org/uniprot/110094
Mouse_swiss_prot_no： Q8BWJ3
Mouse_swiss_link： https://www.uniprot.org/uniprotkb/Q8BWJ3
特異性： This antibody detects endogenous levels of KPB2 at Human/Mouse
組成： Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
來源： Polyclonal, Rabbit,IgG
稀釋： WB 1:500-2000;IHC-p 1:50-300
純化工藝： The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
濃度： 1 mg/ml
儲存： -15°C to -25°C/1 year(Do not lower than -25°C)
說明書： 1
Msds： MSDS_Antibody.pdf
分子量： 136kD
功能： disease:Defects in PHKA2 are the cause of glycogen storage disease type 9A (GSD9A) [MIM:306000]; also known as X-linked liver glycogenosis (XLG). GSD9A is a metabolic disorder resulting in a mild glycogenosis with clinical symptoms that include hepatomegaly, growth retardation, muscle weakness, elevation of glutamate-pyruvate transaminase and glutamate-oxaloacetate transaminase, hypercholesterolemia, hypertriglyceridemia, and fasting hyperketosis. Two subtypes are known: type 1 or classic type, and type 2 or variant type. The variant type is characterized mainly by enlarged liver and growth retardation; patients do not show in vitro enzymatic deficiency of phosphorylase kinase. Unlike other glycogenosis diseases, GSD9A is generally a benign condition. Patients improve with age and are often asymptomatic as adults. Accurate diagnosis is therefore also of prognostic interest.,enzyme regulation:By phosphorylation of various serine residues and by calcium.,function:Phosphorylase b kinase catalyzes the phosphorylation of serine in certain substrates, including troponin I. The alpha chain may bind calmodulin.,pathway:Glycan biosynthesis; glycogen metabolism.,similarity:Belongs to the phosphorylase b kinase regulatory chain family.,subunit:Polymer of 16 chains, four each of alpha, beta, gamma, and delta. Alpha and beta are regulatory chains, gamma is the catalytic chain, and delta is calmodulin.,tissue specificity:Predominantly expressed in liver and other non-muscle tissues.,
相關(guān)產(chǎn)品： RS0001,RS0002,YM3028,YM3029
細(xì)胞定位： Cell membrane ; Lipid-anchor ; Cytoplasmic side .
組織表達(dá)： Predominantly expressed in liver and other non-muscle tissues.
科研貨號： PLA020405

Hunan UPT Biotechnology Co.,Ltd

Website：m.tjdqdt.com Servive hotline :4006916686

E-mail：service@uptbio.com

Address：

Room 402, Building 13, Xinggong International Industrial Park, 100 Guyuan Road, Yuelu District, Changsha City, Hunan Province, China.

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