SAB4300926
Anti-MT-ND1 antibody produced in rabbit
affinity isolated antibody
Synonym(s):
EC 1.6.5.3, MT-ND1, MTND1, NADH dehydrogenase subunit 1, NADH-ubiquinone oxidoreductase chain 1
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About This Item
NACRES:
NA.41
UNSPSC Code:
12352203
Conjugate:
unconjugated
Clone:
polyclonal
Application:
IHC, WB
Citations:
4
biological source
rabbit
conjugate
unconjugated
antibody form
affinity isolated antibody
antibody product type
primary antibodies
clone
polyclonal
form
buffered aqueous solution
mol wt
36 kDa
species reactivity
human
concentration
1.0 mg/mL
technique(s)
immunohistochemistry: 1:50-1:100, western blot: 1:500-1:3000 (Cell Lysate)
isotype
IgG
accession no.
P03886
UniProt accession no.
shipped in
wet ice
storage temp.
−20°C
target post-translational modification
unmodified
Quality Level
Gene Information
human ... MT-ND1(4535)
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General description
Mitochondrially encoded NADH dehydrogenase 1 (MT-ND1) is a component of mitochondrial membrane respiratory chain nicotinamide adenine dinucleotide (NADH) dehydrogenase (Complex I). It comprises eight transmembrane spanning domains and is a conserved protein placed in the hydrophobic arm of the complex I.
Immunogen
Synthesized peptide derived from internal of human MT-ND1.
Biochem/physiol Actions
Mitochondrially encoded NADH dehydrogenase 1 (MT-ND1) participates in ubiquinone binding and proton pumping. Mutations in the MT-ND1 gene are implicated in Leber′s hereditary optic neuropathy (LHON), as well as in mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes (MELAS). Also, some heteroplasmic mutations in the MTND1 gene are associated with an overlap syndrome showing the clinical features associated with LHON and MELAS.
The antibody detects endogenous levels of total MT-ND1 protein.
Features and Benefits
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Physical form
Rabbit IgG in phosphate buffered saline (without Mg2+ and Ca2+), pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.
Disclaimer
Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
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Storage Class
10 - Combustible liquids
wgk
WGK 1
flash_point_f
Not applicable
flash_point_c
Not applicable
Regulatory Information
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Chiara La Morgia et al.
BMC neurology, 14, 116-116 (2014-06-03)
An increasing number of mitochondrial DNA (mtDNA) mutations, mainly in complex I genes, have been associated with variably overlapping phenotypes of Leber's hereditary optic neuropathy (LHON), mitochondrial encephalomyopathy with stroke-like episodes (MELAS) and Leigh syndrome (LS). We here describe the
Mutations of the mitochondrial ND1 gene as a cause of MELAS.
D M Kirby et al.
Journal of medical genetics, 41(10), 784-789 (2004-10-07)
Runyu Guo et al.
Cell, 170(6), 1247-1257 (2017-08-29)
The respiratory megacomplex represents the highest-order assembly of respiratory chain complexes, and it allows mitochondria to respond to energy-requiring conditions. To understand its architecture, we examined the human respiratory chain megacomplex-I2III2IV2 (MCI2III2IV2) with 140 subunits and a subset of associated
Emma L Blakely et al.
European journal of human genetics : EJHG, 13(5), 623-627 (2005-01-20)
Pathogenic point mutations in the mitochondrial MTND1 gene have previously been described in association with two distinct clinical phenotypes -- Leber hereditary optic neuropathy (LHON) and mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes (MELAS). Here we report the first
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