Recombinant人ISCU protein (ab103306)

概述

  • 产品名称Recombinant人ISCU protein
  • 蛋白长度Full length protein

描述

  • 性质Recombinant
  • 来源Escherichia coli
  • 氨基酸序列
    • AccessionQ9H1K1
    • 种属Human
    • 序列MGSSHHHHHHSSGLVPRGSHMYHKKVVDHYENPRNVGSLDKTSKNVGTGL VGAPACGDVMKLQIQVDEKGKIVDARFKTFGCGSAIASSSLATEWVKGKT VEEALTIKNTDIAKELCLPPVKLHCSMLAEDAIKAALADYKLKQEPKKGE AEKK
    • 分子量17 kDa including tags
    • 氨基酸35 to 167
    • 标签His tag N-Terminus

技术指标

Our Abpromise guarantee covers the use of ab103306 in the following tested applications.

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

  • 应用

    SDS-PAGE

    Mass Spectrometry

  • 质谱法
    MALDI-TOF
  • 纯度> 90 % SDS-PAGE.
    ab103306 was purified using conventional chromatography techniques.
  • 形式Liquid
  • Concentration information loading...

制备和贮存

  • 稳定性和存储

    Shipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C or -80°C. Avoid freeze / thaw cycle.

    Preservative: None
    Constituents: 10% Glycerol, 20mM Tris HCl, 2mM DTT, 100mM Sodium chloride, pH 8.0

常规信息

  • 别名
    • 2310020H20Rik
    • HML
    • hnifU
    • Iron sulfur cluster assembly enzyme ISCU mitochondrial
    • Iron sulfur cluster scaffold homolog
    • Iron sulfur cluster scaffold homolog (E. coli)
    • Iron-sulfur cluster assembly enzyme ISCU
    • Iscu
    • IscU iron sulfur cluster scaffold homolog
    • ISCU_HUMAN
    • ISU2
    • MGC74517
    • mitochondrial
    • NIFU
    • NifU like N terminal domain containing
    • NifU like N terminal domain containing protein
    • NifU like protein
    • NifU-like N-terminal domain-containing protein
    • NifU-like protein
    • NIFUN
    • Nitrogen fixation cluster like
    • OTTHUMP00000238760
    • OTTHUMP00000238761
    • OTTHUMP00000238762
    • OTTHUMP00000238764
    • OTTHUMP00000238765
    see all
  • 功能Involved in the assembly or repair of the [Fe-S] clusters present in iron-sulfur proteins. Binds iron.
  • 组织特异性Detected in heart, liver, skeletal muscle, brain, pancreas, kidney, lung and placenta.
  • 疾病相关Defects in ISCU are the cause of myopathy with exercise intolerance Swedish type (MEIS) [MIM:255125]; also known as myopathy with deficiency of succinate dehydrogenase and aconitase or myoglobinuria due to abnormal glycolysis or hereditary myopathy with lactic acidosis (HML). This autosomal recessive metabolic disease is characterized by lifelong severe exercise intolerance, in which minor exertion causes fatigue of active muscles, shortness of breath, and cardiac palpitations in association with lactic acidosis. The biochemical phenotype is characterized by a deficiency in mitochondrial iron-sulfur proteins and impaired muscle oxidative metabolism.
  • 序列相似性Belongs to the nifU family.
  • 细胞定位Cytoplasm. Nucleus and Mitochondrion.
  • Information by UniProt

Recombinant Human ISCU protein 图像

  • 15% SDS-PAGE analysis of 3µg ab103306.

Recombinant Human ISCU protein (ab103306)参考文献

ab103306 has not yet been referenced specifically in any publications.

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