IBA57 Antibody, FITC conjugated

Code CSB-PA722566LC01HU
Size US$166
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Product Details

Full Product Name
Rabbit anti-Homo sapiens (Human) IBA57 Polyclonal antibody
Uniprot No.
Target Names
IBA57
Alternative Names
C1orf69 antibody; CAF17_HUMAN antibody; Chromosome 1 open reading frame 69 antibody; FLJ12734 antibody; FLJ13849 antibody; IBA57 antibody; IBA57 homolog iron sulfur cluster assembly antibody; IBA57 iron sulfur cluster assembly homolog antibody; IBA57 iron sulfur cluster assembly homolog (S. cerevisiae) antibody; IBA57; S. cerevisiae; homolog of antibody; Iron sulfur cluster assembly factor for biotin synthase and aconitase like antibody; iron-sulfur cluster assembly factor for biotin synthase- and aconitase-like mitochondrial proteins; with a mass of 57kDa antibody; Iron-sulfur cluster assembly factor homolog antibody; Mitochondrial proteins with a mass of 57kDa antibody; MMDS3 antibody; Putative transferase C1orf69 mitochondrial antibody; Putative transferase CAF17; mitochondrial antibody; SPG74 antibody
Raised in
Rabbit
Species Reactivity
Human
Immunogen
Recombinant Human Putative transferase CAF17, mitochondrial protein (286-356AA)
Immunogen Species
Homo sapiens (Human)
Conjugate
FITC
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
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.
Gene References into Functions
  1. a patient with infantile-onset optic atrophy and asymptomatic white matter involvement, thus broadening the phenotypic spectrum of biallelic IBA57 mutations. PMID: 30258207
  2. Description of four unrelated patients carrying novel mutations in IBA57; study expands the array of the genotypic variation of IBA57 and delineates the leukodystrophic pattern of IBA57 deficient patients PMID: 27785568
  3. Findings reinforce the suggested specific function of IBA57 in mitochondrial [4Fe-4S] protein maturation and provide additional evidence for its role in hereditary spastic paraplegia PMID: 25609768
  4. Mutation of the iron-sulfur cluster assembly gene IBA57 causes severe myopathy and encephalopathy. PMID: 23462291
Involvement in disease
Multiple mitochondrial dysfunctions syndrome 3 (MMDS3); Spastic paraplegia 74, autosomal recessive (SPG74)
Subcellular Location
Mitochondrion.
Protein Families
GcvT family, CAF17 subfamily
Tissue Specificity
Expressed in skin fibroblasts and skeletal muscle (at protein level).
Database Links

HGNC: 27302

OMIM: 615316

KEGG: hsa:200205

STRING: 9606.ENSP00000355672

UniGene: Hs.237017

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7505 Fannin St., Ste 610, Room 7 (CUBIO Innovation Center), Houston, TX 77054, USA
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