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Recombinant Human Iron-sulfur cluster assembly enzyme ISCU, mitochondrial (ISCU)

ACP10365

Number
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High Purity LevelsPrecision and ReliabilityCustomization Options

Specifications


Cat.No ACP10365 Target NameISCU
FormLyophilized powderExpression SystemCustom Production. Please inquire and provide the desire expression system.
Expression Range35-167Protein LengthFull Length of Mature Protein
Purity>85% (SDS-PAGE)Storage Buffer5%-50% glycerol. Lyophilized powder form: the buffer before lyophilization is Tris/PBS-based buffer, 6% Trehalose, Liquid form: default storage buffer is Tris/PBS-based buffer, pH 8.0.

Immunogen Information


Target SpeciesHumanUniprot IDQ9H1K1
Background Information
  • Uniprot Id

    Q9H1K1

  • Target Species

    Human

  • Target Name

    ISCU

  • Target Full Name

    Iron-sulfur cluster assembly enzyme ISCU

  • Target Function

    Scaffold protein for the de novo synthesis of iron-sulfur (Fe-S) clusters within mitochondria, which is required for maturation of both mitochondrial and cytoplasmic Functions as a cytoplasmic scaffold protein for the de novo synthesis of iron-sulfur clusters in the cytoplasm.

  • Target Involvement

    Myopathy with exercise intolerance Swedish type (MEIS)

  • Target Subcellular Location

    [Isoform 1]: Mitochondrion.; [Isoform 2]: Cytoplasm. Nucleus.

  • Target Protein Families

    NifU family

  • Target Tissue Specificity

    Detected in heart, liver, skeletal muscle, brain, pancreas, kidney, lung and placenta.

  • Target Research Area

    Metabolism, Signal Transduction

  • Target Synonyms

    2310020H20Rik; HML; hnifU; Iron sulfur cluster assembly enzyme ISCU mitochondrial; Iron sulfur cluster scaffold homolog (E. coli); Iron sulfur cluster scaffold homolog; 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

  • Target Background

    This gene encodes a component of the iron-sulfur (Fe-S) cluster scaffold. Fe-S clusters are cofactors that play a role in the function of a diverse set of enzymes, including those that regulate metabolism, iron homeostasis, and oxidative stress response. Alternative splicing results in transcript variants encoding different protein isoforms that localize either to the cytosol or to the mitochondrion. Mutations in this gene have been found in patients with hereditary myopathy with lactic acidosis. A disease-associated mutation in an intron may activate a cryptic splice site, resulting in the production of a splice variant encoding a putatively non-functional protein. A pseudogene of this gene is present on chromosome 1.

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