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Rabbit Anti-SDHC/Gold Conjugated antibody (bs-8530R-Gold)
訂購熱線:400-901-9800
訂購郵箱:sales@bioss.com.cn
訂購QQ:  400-901-9800
技術支持:techsupport@bioss.com.cn
說 明 書: 100ul(10nm  15nm  35nm
100ul/2980.00元
大包裝/詢價
產品編號 bs-8530R-Gold
英文名稱 Rabbit Anti-SDHC/Gold Conjugated antibody
中文名稱 膠體金標記的琥珀酸細胞色素亞基B560抗體
別    名 mitochondrial; QPs1; C560_HUMAN; CYBL; Integral membrane protein CII-3; QPs-1; sdhC; Succinate dehydrogenase complex subunit C; Succinate dehydrogenase cytochrome b560 subunit; Succinate dehydrogenase cytochrome b560 subunit, mitochondrial precursor; Succinate-ubiquinone oxidoreductase cytochrome B large subunit.  
規格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul(10nm  15nm  35nm
研究領域 腫瘤  細胞生物  發育生物學  神經生物學  信號轉導  表觀遺傳學  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 (predicted: Human, )
產品應用 IEM=1:20-200 ICA=1:20-200 ChIP=1:20-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 15kDa
性    狀 Lyophilized or Liquid
濃    度 0.4mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Succinate dehydrogenase complex subunit C/SDHC
亞    型 IgG
純化方法 affinity purified by Protein A
儲 存 液 0.02M TBS(pH8.2) with 1% BSA, 0.03% Proclin300.
保存條件 Store at 2-8 oC for 3-6 months. Avoid repeated freeze/thaw cycles.
產品介紹 background:
Membrane-anchoring subunit of succinate dehydrogenase (SDH) that is involved in complex II of the mitochondrial electron transport chain and is responsible for transferring electrons from succinate to ubiquinone.

Function:
Membrane-anchoring subunit of succinate dehydrogenase (SDH) that is involved in complex II of the mitochondrial electron transport chain and is responsible for transferring electrons from succinate to ubiquinone (coenzyme Q).

Subunit:
Component of complex II composed of four subunits: the flavoprotein (FP) SDHA, iron-sulfur protein (IP) SDHB, and a cytochrome b560 composed of SDHC and SDHD.

Subcellular Location:
Mitochondrion inner membrane.

DISEASE:
Defects in SDHC are the cause of paragangliomas type 3 (PGL3) [MIM:605373]. A neural crest tumor usually derived from the chromoreceptor tissue of a paraganglion. Paragangliomas are most commonly located in the head and neck region, specifically at the carotid bifurcation, the jugular foramen, the vagal nerve, and in the middle ear.
Defects in SDHC are a cause of paraganglioma and gastric stromal sarcoma (PGGSS) [MIM:606864]; also known as Carney-Stratakis syndrome. Gastrointestinal stromal tumors may be sporadic or inherited in an autosomal dominant manner, alone or as a component of a syndrome associated with other tumors, such as in the context of neurofibromatosis type 1 (NF1). Patients have both gastrointestinal stromal tumors and paragangliomas. Susceptibility to the tumors was inherited in an apparently autosomal dominant manner, with incomplete penetrance.

Similarity:
Belongs to the cytochrome b560 family.

Database links:

Entrez Gene: 327696 Cow

Entrez Gene: 6391 Human

Entrez Gene: 66052 Mouse

Entrez Gene: 100524676 Pig

Entrez Gene: 289217 Rat

Omim: 602413 Human

SwissProt: P35720 Cow

SwissProt: Q99643 Human

SwissProt: Q9CZB0 Mouse

SwissProt: D0VWV4 Pig

Unigene: 444472 Human

Unigene: 198138 Mouse

Unigene: 1698 Rat



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

Involvement in disease:Defects in SDHC are the cause of hereditary paragangliomas type 3 (PGL3) [MIM:605373]; also known as autosomal dominant non-chromaffin paragangliomas type 3. Non-chromaffin paragangliomas are usually benign, neural crest derived tumors of parasympathetic ganglia.
Defects in SDHC are a cause of paraganglioma and gastric stromal sarcoma (PGGSS) ; also known as Carney-Stratakis syndrome. Gastrointestinal stromal tumors may be sporadic or inherited in an autosomal dominant manner, alone or as a component of a syndrome associated with other tumors, such as in the context of neurofibromatosis type 1 (NF1). Patients have both gastrointestinal stromal tumors and paragangliomas. Susceptibility to the tumors was inherited in an apparently autosomal dominant manner, with incomplete penetrance.
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