Recombinant人SDHD protein (ab116859)

概述

描述

  • 性质
    Recombinant
  • 来源
    Wheat germ
  • 氨基酸序列
    • Accession
    • 种属
      Human
    • 序列
      MAVLWRLSAVCGALGGRALLLRTPVVRPAHISAFLQDRPIPEWCGVQHIH LSPSHHSGSKAASLHWTSERVVSVLLLGLLPAAYLNPCSAMDYSLAAALT LHGHWGLGQVVTDYVHGDALQKAAKAGLLALSALTFAGLCYFNYHDVGIC KAVAMLWKL
    • 分子量
      44 kDa including tags
    • 氨基酸
      1 to 159

技术指标

Our Abpromise guarantee covers the use of ab116859 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

  • 应用

    ELISA

    SDS-PAGE

    Western blot

  • 形式
    Liquid
  • 补充说明
    Protein concentration is above or equal to 0.05 mg/ml. This protein is best used within three months from the date of receipt.
  • Concentration information loading...

制备和贮存

  • 稳定性和存储

    Shipped on dry ice. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.

    pH: 8.00
    Constituents: 0.3% Glutathione, 0.79% Tris HCl

常规信息

  • 别名
    • CBT1
    • CII 4
    • CII-4
    • CII4
    • CWS3
    • CybS
    • DHSD_HUMAN
    • mitochondrial
    • OTTHUMP00000234720
    • OTTHUMP00000234721
    • OTTHUMP00000234722
    • OTTHUMP00000234723
    • OTTHUMP00000234724
    • OTTHUMP00000234725
    • OTTHUMP00000234726
    • PGL
    • PGL1
    • QPs3
    • SDH4
    • sdhD
    • Succinate dehydrogenase [ubiquinone] cytochrome b small subunit
    • Succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial
    • Succinate dehydrogenase complex subunit D
    • Succinate dehydrogenase complex, subunit D, integral membrane protein
    • Succinate dehydrogenase ubiquinone cytochrome B small subunit
    • Succinate ubiquinone oxidoreductase cytochrome b small subunit
    • Succinate ubiquinone reductase membrane anchor subunit
    • Succinate-ubiquinone oxidoreductase cytochrome b small subunit
    • Succinate-ubiquinone reductase membrane anchor subunit
    see all
  • 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).
  • 通路
    Carbohydrate metabolism; tricarboxylic acid cycle.
  • 疾病相关
    Defects in SDHD are a cause of hereditary paragangliomas type 1 (PGL1) [MIM:168000]; also known as familial non-chromaffin paragangliomas type 1. Paragangliomas refer to rare and mostly benign tumors that arise from any component of the neuroendocrine system. PGL1 is a rare autosomal dominant disorder which is characterized by the development of mostly benign, highly vascular, slowly growing tumors in the head and neck. In the head and neck region, the carotid body is the largest of all paraganglia and is also the most common site of the tumors. Penetrance of PGL1 is incomplete when the disease is transmitted through fathers. No disease phenotype is transmitted maternally.
    Defects in SDHD are a cause of susceptibility to pheochromocytoma (PCC) [MIM:171300]. A catecholamine-producing tumor of chromaffin tissue of the adrenal medulla or sympathetic paraganglia. The cardinal symptom, reflecting the increased secretion of epinephrine and norepinephrine, is hypertension, which may be persistent or intermittent.
    Defects in SDHD may be a cause of susceptibility to intestinal carcinoid tumor (ICT) [MIM:114900]. A yellow, well-differentiated, circumscribed tumor that arises from enterochromaffin cells in the small intestine or, less frequently, in other parts of the gastrointestinal tract.
    Defects in SDHD are a cause of paraganglioma and gastric stromal sarcoma (PGGSS) [MIM:606864]; also called 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.
    Defects in SDHD are a cause of Cowden-like syndrome (CWDLS) [MIM:612359]. Cowden-like syndrome is a cancer predisposition syndrome associated with elevated risk for tumors of the breast, thyroid, kidney and uterus.
  • 序列相似性
    Belongs to the CybS family.
  • 细胞定位
    Mitochondrion inner membrane.
  • Information by UniProt

图片

  • 12.5% SDS-PAGE showing ab116859 at approximately 43.56kDa and stained with Coomassie Blue.

文献

ab116859 has not yet been referenced specifically in any publications.

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