CNGA1 Antibody, Biotin conjugated

說明書

中文名稱：

CNGA1兔多克隆抗體, Biotin偶聯
貨號：

CSB-PA005642LD01HU
規格：

￥880
其他：

產品詳情
產品評價
相關產品
靶點詳情

產品詳情

產品名稱：

Rabbit anti-Homo sapiens (Human) CNGA1 Polyclonal antibody
Uniprot No.：

P29973
基因名：

CNGA1
別名：

CNGA1; CNCG; CNCG1; cGMP-gated cation channel alpha-1; Cyclic nucleotide-gated cation channel 1; Cyclic nucleotide-gated channel alpha-1; CNG channel alpha-1; CNG-1; CNG1; Cyclic nucleotide-gated channel, photoreceptor; Rod photoreceptor cGMP-gated channel subunit alpha
宿主：

Rabbit
反應種屬：

Human
免疫原：

Recombinant Human cGMP-gated cation channel alpha-1 protein (586-690AA)
免疫原種屬：

Homo sapiens (Human)
標記方式：

Biotin
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
產品提供形式：

Liquid
應用范圍：

ELISA
Protocols：

ELISA Protocol
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.

產品評價

靶點詳情

功能：

Subunit of the rod cyclic GMP-gated cation channel, which is involved in the final stage of the phototransduction pathway. When light hits rod photoreceptors, cGMP concentrations decrease causing rapid closure of CNGA1/CNGB1 channels and, therefore, hyperpolarization of the membrane potential.
基因功能參考文獻：
1. Compound heterozygous mutations (NM000087, p.Y82X and p.L89fs) in exon 6 of CNGA1are pathogenic mutations in this Chinese family affected with autosomal recessive retinitis pigmentosa. Of which, p.Y82X is firstly reported in patient with autosomal recessive retinitis pigmentosa. PMID: 27391953
2. The mut CNGA1 p.(G513R) protein was largely retained inside the cell rather than being targeted to the plasma membrane, suggesting the absence of cGMP-gated cation channels in the plasma membrane PMID: 26802146
3. CNGA1 mutations are one of the most frequent autosomal recessive retinitis pigmentosa-causing mutations in the Japanese patients. PMID: 25268133
4. Insulin receptor (IR), an integral membrane protein, directly phosphorylates the CNGA1 subunit of CNG channels that in turn affects the function of these channels negatively. CNGA1 is a direct substrate of IR. PMID: 23032687
5. Novel homozygous mutation R28X truncates CNGA1 very prematurely in patients with autosomal recessive retinitis pigmentosa. PMID: 12362048
6. isolated an endogenous transcript (anti-CNG1) that is antisense to CNG1 mRNA; data suggest the suppression of CNG(alpha)1 expression by anti-CNG1 may play an important role in neuronal functions, especially in synaptic plasticity and cortical development PMID: 12388767
7. The heteromeric cyclic nucleotide-gated channel adopts a 3A:1B stoichiometry PMID: 12432397
8. The autosomal recessive retinitis pigmentosa in this family is caused by a mutation in CNGA1 gene. PMID: 15570217
9. Expressed in a stage- and cell-specific manner in the rat testis. PMID: 15713832
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相關疾病：

Retinitis pigmentosa 49 (RP49)
亞細胞定位：

Cell membrane; Multi-pass membrane protein.
蛋白家族：

Cyclic nucleotide-gated cation channel (TC 1.A.1.5) family, CNGA1 subfamily
組織特異性：

Rod cells in the retina.
數據庫鏈接：

HGNC: 2148

OMIM: 123825

KEGG: hsa:1259

STRING: 9606.ENSP00000384264

UniGene: Hs.1323

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