NDUFA1 Antibody

Code CSB-PA220820
Size US$166
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Image
  • The image on the left is immunohistochemistry of paraffin-embedded Human thyroid cancer tissue using CSB-PA220820(NDUFA1 Antibody) at dilution 1/70, on the right is treated with fusion protein. (Original magnification: ×200)
  • The image on the left is immunohistochemistry of paraffin-embedded Human colon cancer tissue using CSB-PA220820(NDUFA1 Antibody) at dilution 1/70, on the right is treated with fusion protein. (Original magnification: ×200)
  • Gel: 10%SDS-PAGE, Lysate: 40 μg, Lane: Hela cells, Primary antibody: CSB-PA220820(NDUFA1 Antibody) at dilution 1/400, Secondary antibody: Goat anti rabbit IgG at 1/8000 dilution, Exposure time: 10 seconds
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Product Details

Uniprot No.
Target Names
NDUFA1
Alternative Names
NDUFA1; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1; Complex I-MWFE; CI-MWFE; NADH-ubiquinone oxidoreductase MWFE subunit
Raised in
Rabbit
Species Reactivity
Human
Immunogen
Fusion protein of Human NDUFA1
Immunogen Species
Homo sapiens (Human)
Conjugate
Non-conjugated
Isotype
IgG
Purification Method
Antigen affinity purification
Concentration
It differs from different batches. Please contact us to confirm it.
Buffer
-20°C, pH7.4 PBS, 0.05% NaN3, 40% Glycerol
Form
Liquid
Tested Applications
ELISA,WB,IHC
Recommended Dilution
Application Recommended Dilution
ELISA 1:2000-1:5000
WB 1:500-1:2000
IHC 1:100-1:300
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
Accessory subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I), that is believed not to be involved in catalysis. Complex I functions in the transfer of electrons from NADH to the respiratory chain. The immediate electron acceptor for the enzyme is believed to be ubiquinone.
Gene References into Functions
  1. Results from a study on gene expression variability markers in early-stage human embryos shows that NDUF1A is a putative expression variability marker for the 3-day, 8-cell embryo stage. PMID: 26288249
  2. The gene signature of OPA1, CTSA, NDUFA1, STK10 and PRDX1 was able to identify patients post-implant with a sensitivity of 91% and a specificity of 86% in discrimination between post-implant group and healthy controls. PMID: 27177495
  3. Fanconi anemia complementation group A mutants show defective respiration through Complex I, diminished ATP production and metabolic sufferance with an increased AMP/ATP ratio. PMID: 23791750
  4. Heterozygous mutation in the X chromosomal NDUFA1 gene in a girl with complex I deficiency PMID: 21596602
  5. Mutations in the NDUFA1 gene is linked to a delayed mitochondrial network recovery in OXPHOS disorders. PMID: 20153825
  6. Species-specific and mutant MWFE proteins effect on the assembly of a functional mammalian mitochondrial complex I. PMID: 11937507
  7. A family-based association study finds that the NDUFA1 gene is unlikely to harbor a major visual loss susceptibility locus for Leber hereditary optic neuropathy. PMID: 12084895
  8. Oxidative stress and partial deficiencies of mitochondrial complex I are key factors in the pathogenesis of Parkinson's disease. (REVIEW) PMID: 15038604
  9. Suppression of NDUFA1 expression could represent a key pathogenic mechanism in the development of basal cell carcinoma PMID: 15854127
  10. Two novel p.Gly8Arg and p.Arg37Ser hemizygous mutations in NDUFA1 were identified in two unrelated male patients presenting with Leigh's syndrome and with myoclonic epilepsy and developmental delay. PMID: 17262856
  11. hypothesize that the novel G32R mutation in NDUFA1 is causing complex I deficiency either by itself or in synergy with additional mtDNA variants PMID: 19185523

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Involvement in disease
Mitochondrial complex I deficiency (MT-C1D)
Subcellular Location
Mitochondrion inner membrane; Single-pass membrane protein; Matrix side.
Protein Families
Complex I NDUFA1 subunit family
Tissue Specificity
Primarily expressed in heart and skeletal muscle.
Database Links

HGNC: 7683

OMIM: 252010

KEGG: hsa:4694

STRING: 9606.ENSP00000360492

UniGene: Hs.534168

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