Overview

  • Product nameRecombinant Human ISCU protein
  • Protein lengthFull length protein

Description

  • NatureRecombinant
  • SourceEscherichia coli
  • Amino Acid Sequence
    • AccessionQ9H1K1
    • SpeciesHuman
    • SequenceMGSSHHHHHHSSGLVPRGSHMYHKKVVDHYENPRNVGSLDKTSKNVGTGL VGAPACGDVMKLQIQVDEKGKIVDARFKTFGCGSAIASSSLATEWVKGKT VEEALTIKNTDIAKELCLPPVKLHCSMLAEDAIKAALADYKLKQEPKKGE AEKK
    • Molecular weight17 kDa including tags
    • Amino acids35 to 167
    • TagsHis tag N-Terminus

Specifications

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.

  • Applications

    SDS-PAGE

    Mass Spectrometry

  • Mass spectrometry
    MALDI-TOF
  • Purity> 90 % SDS-PAGE.
    ab103306 was purified using conventional chromatography techniques.
  • FormLiquid
  • Concentration information loading...

Preparation and Storage

  • Stability and Storage

    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

General Info

  • Alternative names
    • 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
  • FunctionInvolved in the assembly or repair of the [Fe-S] clusters present in iron-sulfur proteins. Binds iron.
  • Tissue specificityDetected in heart, liver, skeletal muscle, brain, pancreas, kidney, lung and placenta.
  • Involvement in diseaseDefects 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.
  • Sequence similaritiesBelongs to the nifU family.
  • Cellular localizationCytoplasm. Nucleus and Mitochondrion.
  • Information by UniProt

Recombinant Human ISCU protein images

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

References for Recombinant Human ISCU protein (ab103306)

ab103306 has not yet been referenced specifically in any publications.

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