POMK Antibody, FITC conjugated
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中文名稱：POMK兔多克隆抗體, FITC偶聯(lián)
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貨號(hào)：CSB-PA863985LC01HU
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規(guī)格：￥880
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其他：
產(chǎn)品詳情
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產(chǎn)品名稱：Rabbit anti-Homo sapiens (Human) POMK Polyclonal antibody
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Uniprot No.：
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基因名：POMK
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別名：FLJ23356 antibody; MDDGA12 antibody; POMK antibody; Probable inactive protein kinase-like protein SgK196 antibody; Protein kinase like protein SgK196 antibody; Protein kinase-like protein SgK196 antibody; Protein O-mannose kinase antibody; SG196_HUMAN antibody; SGK196 antibody; Sugen kinase 196 antibody
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宿主：Rabbit
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反應(yīng)種屬：Human
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免疫原：Recombinant Human Protein O-mannose kinase protein (44-350AA)
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免疫原種屬：Homo sapiens (Human)
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標(biāo)記方式：FITC
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克隆類型：Polyclonal
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抗體亞型：IgG
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純化方式：>95%, Protein G purified
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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：Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, PH 7.4 -
產(chǎn)品提供形式：Liquid
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儲(chǔ)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(chǎn)品評(píng)價(jià)
相關(guān)產(chǎn)品
靶點(diǎn)詳情
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功能：Protein O-mannose kinase that specifically mediates phosphorylation at the 6-position of an O-mannose of the trisaccharide (N-acetylgalactosamine (GalNAc)-beta-1,3-N-acetylglucosamine (GlcNAc)-beta-1,4-mannose) to generate phosphorylated O-mannosyl trisaccharide (N-acetylgalactosamine-beta-1,3-N-acetylglucosamine-beta-1,4-(phosphate-6-)mannose). Phosphorylated O-mannosyl trisaccharide is a carbohydrate structure present in alpha-dystroglycan (DAG1), which is required for binding laminin G-like domain-containing extracellular proteins with high affinity. Only shows kinase activity when the GalNAc-beta-3-GlcNAc-beta-terminus is linked to the 4-position of O-mannose, suggesting that this disaccharide serves as the substrate recognition motif.
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基因功能參考文獻(xiàn)：
- POMK mutations disrupt muscle development leading to a spectrum of neuromuscular presentations. PMID: 24925318
- Homozygous truncating mutations in POMK lead to congenital muscular dystrophies with secondary merosin deficiency, hypomyelination and intellectual disability. PMID: 24556084
- These findings suggest how mutations in GTDC2, B3GALNT2, and SGK196 disrupt dystroglycan receptor function and lead to congenital muscular dystrophy. PMID: 23929950
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相關(guān)疾?。?/div>Muscular dystrophy-dystroglycanopathy congenital with brain and eye anomalies A12 (MDDGA12); Muscular dystrophy-dystroglycanopathy limb-girdle C12 (MDDGC12)亞細(xì)胞定位：Endoplasmic reticulum membrane; Single-pass type II membrane protein.蛋白家族：Protein kinase superfamily, Ser/Thr protein kinase family, STKL subfamily組織特異性：Highest expression is observed in brain, skeletal muscle, kidney and heart in fetal and adult tissues.數(shù)據(jù)庫鏈接：
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