SDHC Recombinant Rabbit mAb (一抗) - WB,IHC-P,IHC-F,IF | Bioss

Rrmab?兔单抗
2026-05-01~2026-06-30,AB2605
SDHC Recombinant Rabbit mAb (一抗) - WB,IHC-P,IHC-F,IF | Bioss
货号:bsm-62867R
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概述

产品编号
bsm-62867R
产品类型
重组兔单抗
英文名称
SDHC Recombinant Rabbit mAb
中文名称
琥珀酸细胞色素亚基B560重组兔单抗
英文别名
CYB560; CYBL; PGL3; PPGL3; QPS1; SDH3; 0610010E03Rik; C560_BOVIN; SDHC; Integral membrane protein CII-3; Malate dehydrogenase [quinone] cytochrome b560 subunit; QPs-1 (QPs1); Succinate dehydrogenase complex subunit C; C560_HUMAN; Succinate-ubiquinone oxidoreductase cytochrome B large subunit (CYBL); C560_MOUSE; C560_PIG; succinate dehydrogenase complex, subunit C, integral membrane protein, 15kD; succinate dehydrogenase complex, subunit C, integral membrane protein, 15kDa; succinate dehydrogenase cytochrome b560 subunit; succinate dehydrgenase cytochrome b; large subunit of cytochrome b
抗体来源
Rabbit
免疫原
A synthesized peptide derived from human SDHC: 10-69/169
亚型
IgG
性状
Liquid
纯化方法
affinity purified by Protein A
克隆类型
Recombinant
理论分子量
19 kDa
检测分子量
17 kDa
储存液
10mM phosphate buffered saline(pH 7.4) with 150mM sodium chloride, 0.05% BSA, 0.02% Proclin300 and 50% glycerol.
SWISS
Gene ID
保存条件
Store at 4℃ for short term. Store at -20℃ for long term. Avoid repeated freeze/thaw cycles.
注意事项
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
产品介绍
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.
背景资料
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.
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产品应用

应用已检合格种属预测种属推荐稀释比例
WBHuman, Mouse, Rat1:500-2000
IHC-PHuman, Mouse, Rat1:100-500
IHC-FHuman, Mouse, Rat1:100-500
IFHuman, Mouse, Rat1:100-500

交叉反应

交叉反应: Human, Mouse, Rat

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靶标

基因名
SDHC
蛋白名
Succinate dehydrogenase cytochrome b560 subunit, mitochondrial
亚基
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.
亚细胞定位
Mitochondrion inner membrane.
疾病
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.
相似性
Belongs to the cytochrome b560 family.
功能
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).

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