CHRND Antibody, FITC conjugated

中文名稱：

CHRND兔多克隆抗體, FITC偶聯(lián)
貨號：

CSB-PA005399LC01HU
規(guī)格：

￥880
其他：

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產(chǎn)品名稱：

Rabbit anti-Homo sapiens (Human) CHRND Polyclonal antibody
Uniprot No.：

Q07001
基因名：

CHRND
別名：

Acetylcholine receptor delta subunit antibody; Acetylcholine receptor subunit delta antibody; ACHD_HUMAN antibody; ACHRD antibody; Cholinergic receptor, nicotinic, delta polypeptide antibody; CHRND antibody; CMS2A antibody; FCCMS antibody; Nicotinic acetylcholine receptor delta polypeptide precursor antibody; SCCMS antibody
宿主：

Rabbit
反應(yīng)種屬：

Human
免疫原：

Recombinant Human Acetylcholine receptor subunit delta protein (334-471AA)
免疫原種屬：

Homo sapiens (Human)
標(biāo)記方式：

FITC
克隆類型：

Polyclonal
抗體亞型：

IgG
純化方式：

>95%, Protein G purified
濃度：

It differs from different batches. Please contact us to confirm it.
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
儲存條件：

Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
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.
用途：

For Research Use Only. Not for use in diagnostic or therapeutic procedures.

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功能：

After binding acetylcholine, the AChR responds by an extensive change in conformation that affects all subunits and leads to opening of an ion-conducting channel across the plasma membrane.
基因功能參考文獻(xiàn)：
1. Data suggest mutation in invariant Cys-loop of CHRND (D140N) observed in muscle of one patient (11 y/o girl) with congenital myasthenia (w/ severe muscle weakness) alters conformation of ligand/acetylcholine binding site and receptor functionality. PMID: 26698174
2. This study showed that a single mutation of the delta subunit, L332P, allows the synapse in slow muscles to function but renders those in fast muscles almost nonfunctional. PMID: 25080583
3. Results describe the effects of a point mutation in the AChR delta subunit from a congenital myasthenia patient. PMID: 18398509
相關(guān)疾病：

Multiple pterygium syndrome, lethal type (LMPS); Myasthenic syndrome, congenital, 3A, slow-channel (CMS3A); Myasthenic syndrome, congenital, 3B, fast-channel (CMS3B); Myasthenic syndrome, congenital, 3C, associated with acetylcholine receptor deficiency (CMS3C)
亞細(xì)胞定位：

Cell junction, synapse, postsynaptic cell membrane; Multi-pass membrane protein. Cell membrane; Multi-pass membrane protein.
蛋白家族：

Ligand-gated ion channel (TC 1.A.9) family, Acetylcholine receptor (TC 1.A.9.1) subfamily, Delta/CHRND sub-subfamily
數(shù)據(jù)庫鏈接：

HGNC: 1965

OMIM: 100720

KEGG: hsa:1144

STRING: 9606.ENSP00000258385

UniGene: Hs.156289

CHRND Antibody, FITC conjugated

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