製品の概要

  • 製品名Recombinant Human ISCU protein
  • タンパク質長Full length protein

製品の詳細

  • 由来Recombinant
  • 由来Escherichia coli
  • アミノ酸配列
    • アクセッション番号Q9H1K1
    • 生物種Human
    • 配列MGSSHHHHHHSSGLVPRGSHMYHKKVVDHYENPRNVGSLDKTSKNVGTGL VGAPACGDVMKLQIQVDEKGKIVDARFKTFGCGSAIASSSLATEWVKGKT VEEALTIKNTDIAKELCLPPVKLHCSMLAEDAIKAALADYKLKQEPKKGE AEKK
    • 分子量17 kDa including tags
    • 領域35 to 167
    • タグHis tag N-Terminus

特性

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.

  • アプリケーション

    SDS-PAGE

    Mass Spectrometry

  • 質量分析
    MALDI-TOF
  • 精製度> 90 % SDS-PAGE.
    ab103306 was purified using conventional chromatography techniques.
  • 製品の状態Liquid
  • Concentration information loading...

前処理および保存

  • 保存方法および安定性

    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

関連情報

  • 別名
    • 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
  • 機能Involved in the assembly or repair of the [Fe-S] clusters present in iron-sulfur proteins. Binds iron.
  • 組織特異性Detected in heart, liver, skeletal muscle, brain, pancreas, kidney, lung and placenta.
  • 関連疾患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.
  • 配列類似性Belongs to the nifU family.
  • 細胞内局在Cytoplasm. Nucleus and Mitochondrion.
  • Information by UniProt

Recombinant Human ISCU protein 画像

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

Recombinant Human ISCU protein (ab103306) 使用論文

ab103306 has not yet been referenced specifically in any publications.

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