NDUFS2 Antibody, Biotin conjugated

Code CSB-PA015661LD01HU
Size US$166
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Product Details

Full Product Name
Rabbit anti-Homo sapiens (Human) NDUFS2 Polyclonal antibody
Uniprot No.
Target Names
NDUFS2
Alternative Names
CI 49 antibody; CI 49kD antibody; CI-49kD antibody; Complex 1, mitochondrial respiratory chain, 49 KD subunit antibody; Complex I 49kD antibody; Complex I 49kDa subunit antibody; Complex I-49kD antibody; mitochondrial antibody; NADH dehydrogenase (ubiquinone) Fe S protein 2 49kDa antibody; NADH dehydrogenase (ubiquinone) Fe S protein 2, 49kDa (NADH coenzyme Q reductase) antibody; NADH dehydrogenase [ubiquinone] iron sulfur protein 2, mitochondrial antibody; NADH dehydrogenase [ubiquinone] iron-sulfur protein 2 antibody; NADH ubiquinone oxidoreductase 49 kDa subunit antibody; NADH ubiquinone oxidoreductase NDUFS2 subunit antibody; NADH-ubiquinone oxidoreductase 49 kDa subunit antibody; NADH:ubiquinone oxidoreductase core subunit S2 antibody; Ndufs2 antibody; NDUS2_HUMAN antibody
Raised in
Rabbit
Species Reactivity
Human
Immunogen
Recombinant Human NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial protein (297-427AA)
Immunogen Species
Homo sapiens (Human)
Conjugate
Biotin
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
Core subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I) which catalyzes electron transfer from NADH through the respiratory chain, using ubiquinone as an electron acceptor. Essential for the catalytic activity of complex I. Essential for the assembly of complex I. Redox-sensitive, critical component of the oxygen-sensing pathway in the pulmonary vasculature which plays a key role in acute pulmonary oxygen-sensing and hypoxic pulmonary vasoconstriction. Plays an important role in carotid body sensing of hypoxia. Essential for glia-like neural stem and progenitor cell proliferation, differentiation and subsequent oligodendrocyte or neuronal maturation.
Gene References into Functions
  1. compound heterozygosity for severe and hypomorphic NDUFS2 mutations can cause non-syndromic hereditary optic neuropathy. PMID: 28031252
  2. NDUFAF7 methylates arginine 85 in the NDUFS2 subunit of human complex I. PMID: 24089531
  3. study describes clinical, radiological, biochemical and molecular data of 6 patients with Leigh syndrome with novel mutations in NDUFV1 and NDUFS2; 2 siblings were compound heterozygotes for an undescribed E104A mutation in NDUFS2 PMID: 23266820
  4. The NDUFS2 mutation affects complex I enzymatic function. PMID: 22036843
  5. Our results confirm that NDUFS2 is a mutational hotspot in Caucasian children with isolated complex I deficiency and recommend the routine diagnostic investigation of this gene in patients with Leigh or Leigh-like phenotypes. PMID: 20819849

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Involvement in disease
Mitochondrial complex I deficiency (MT-C1D)
Subcellular Location
Mitochondrion inner membrane; Peripheral membrane protein; Matrix side.
Protein Families
Complex I 49 kDa subunit family
Database Links

HGNC: 7708

OMIM: 252010

KEGG: hsa:4720

STRING: 9606.ENSP00000356972

UniGene: Hs.173611

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