Recombinant Human Iron-sulfur cluster assembly enzyme ISCU, mitochondrial (ISCU) | CSB-EP887955HU(A4)

Recombinant Human Iron-sulfur cluster assembly enzyme ISCU, mitochondrial (ISCU) | CSB-EP887955HU(A4) | Cusabio

Alternative Name(s): NifU-like N-terminal domain-containing protein NifU-like protein

Gene Names: ISCU

Research Areas: Signal Transduction

Organism: Homo sapiens (Human)

AA Sequence: MAAAGAFRLRRAASALLLRSPRLPARELSAPARLYHKKVVDHYENPRNVGSLDKTSKNVGTGLVGAPACGDVMKLQIQVDEKGKIVDARFKTFGCGSAIASSSLATEWVKGKTVEEALTIKNTDIAKELCLPPVKLHCSMLAEDAIKAALADYKLKQEPKKGEAEKK

Source: E.coli

Tag Info: N-terminal GST-tagged

Expression Region: 1-167aa

Sequence Info: Full Length

MW: 45.0 kDa

Purity: Greater than 90% as determined by SDS-PAGE.

Relevance: 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 [2Fe-2S] and [4Fe-4S] proteins. First, a [2Fe-2S] cluster is transiently assembled on the scaffold protein ISCU. In a second step, the cluster is released from ISCU, transferred to a glutaredoxin GLRX5, followed by the formation of mitochondrial [2Fe-2S] proteins, the synthesis of [4Fe-4S] clusters and their target-specific insertion into the recipient apoproteins. Cluster assembly on ISCU depends on the function of the cysteine desulfurase complex NFS1-LYRM4/ISD11, which serves as the sulfur donor for cluster synthesis, the iron-binding protein frataxin as the putative iron donor, and the electron transfer chain comprised of ferredoxin reductase and ferredoxin, which receive their electrons from NADH

Reference: "Distinct iron-sulfur cluster assembly complexes exist in the cytosol and mitochondria of human cells." Tong W.-H., Rouault T. EMBO J. 19:5692-5700(2000)

Storage: The shelf life is related to many factors, storage state, buffer ingredients, storage temperature and the stability of the protein itself. Generally, the shelf life of liquid form is 6 months at -20?/-80?. The shelf life of lyophilized form is 12 months at -20?/-80?.

Notes: Repeated freezing and thawing is not recommended. Store working aliquots at 4? for up to one week.

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 [2Fe-2S] and [4Fe-4S] proteins

Involvement in disease: Myopathy with exercise intolerance Swedish type (MEIS)

Subcellular Location: Isoform 1: Mitochondrion, SUBCELLULAR LOCATION: Isoform 2: Cytoplasm, Nucleus

Protein Families: NifU family

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

Paythway:

Form: Liquid or Lyophilized powder

Buffer: If the delivery form is liquid, the default storage buffer is Tris/PBS-based buffer, 5%-50% glycerol. If the delivery form is lyophilized powder, the buffer before lyophilization is Tris/PBS-based buffer, 6% Trehalose, pH 8.0.

Reconstitution: We recommend that this vial be briefly centrifuged prior to opening to bring the contents to the bottom. Please reconstitute protein in deionized sterile water to a concentration of 0.1-1.0 mg/mL.We recommend to add 5-50% of glycerol (final concentration) and aliquot for long-term storage at -20?/-80?. Our default final concentration of glycerol is 50%. Customers could use it as reference.

Uniprot ID: Q9H1K1

HGNC Database Link: HGNC

UniGene Database Link: UniGene

KEGG Database Link: KEGG

STRING Database Link: STRING

OMIM Database Link: OMIM