ISCA1 Antibody, HRP conjugated

Code CSB-PA887126LB01HU
Size US$166
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Product Details

Full Product Name
Rabbit anti-Homo sapiens (Human) ISCA1 Polyclonal antibody
Uniprot No.
Target Names
ISCA1
Alternative Names
GK004 antibody; HESB-like domain-containing protein 2 antibody; hIscA antibody; Iron sulfur assembly protein IscA antibody; Iron-sulfur assembly protein IscA antibody; Iron-sulfur cluster assembly 1 homolog antibody; Iron-sulfur cluster assembly 1 homolog, mitochondrial antibody; ISCA1 antibody; ISCA1_HUMAN antibody; mitochondrial antibody
Raised in
Rabbit
Species Reactivity
Human
Immunogen
Recombinant Human Iron-sulfur cluster assembly 1 homolog, mitochondrial protein (67-122AA)
Immunogen Species
Homo sapiens (Human)
Conjugate
HRP
Clonality
Polyclonal
Isotype
IgG
Purification Method
>95%, Protein G purified
Concentration
It differs from different batches. Please contact us to confirm it.
Buffer
Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
Form
Liquid
Tested Applications
ELISA
Protocols
Troubleshooting and FAQs
Storage
Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
Lead Time
Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.

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Target Background

Function
Involved in the maturation of mitochondrial 4Fe-4S proteins functioning late in the iron-sulfur cluster assembly pathway. Probably involved in the binding of an intermediate of Fe/S cluster assembly.
Gene References into Functions
  1. the phenotype observed in all affected subjects with the ISCA1 pathogenic variant is similar to that previously described in all four types of multiple mitochondrial dysfunctions syndromes. PMID: 28356563
  2. The structural plasticity of the dimeric state of the [2Fe-2S] bound form of human GRX5 is the crucial factor that allows an efficient cluster transfer to the partner proteins human ISCA1 and ISCA2 by a specific protein-protein recognition mechanism. PMID: 24733926
  3. The data presented here show that the Isu1 suppressor mimics the frataxin effects on Nfs1, explaining the bypassing activity. PMID: 24217246
  4. ISCA1, ISCA2, and IBA57 were depleted by RNA interference. Depleted cells contained massively swollen and enlarged mitochondria that were virtually devoid of cristae membranes, demonstrating the importance of these proteins for mitochondrial biogenesis. PMID: 22323289
  5. Iron-binding activity of human iron-sulfur cluster assembly protein hIscA1 PMID: 20302570
  6. screening a human brain cDNA expression library with serum from a patient suffering from the autoimmune Sjogren's syndrome yielded a gene product called hIscA implicated in iron-sulfur cluster biogenesis PMID: 15262227
  7. Required for functional heme biosynthesis in erythroid cells. PMID: 19656490

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Involvement in disease
Multiple mitochondrial dysfunctions syndrome 5 (MMDS5)
Subcellular Location
Mitochondrion.
Protein Families
HesB/IscA family
Tissue Specificity
Detected in cerebellum, kidney and heart.
Database Links

HGNC: 28660

OMIM: 611006

KEGG: hsa:81689

STRING: 9606.ENSP00000365159

UniGene: Hs.449291

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