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SAB4300926

Sigma-Aldrich

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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100 μL
$357.00

About This Item

UNSPSC Code:
12352203
NACRES:
NA.41

$357.00


Usually ships in 5 business days. (Orders outside of US, please allow an additional 1-2 weeks for delivery)

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Get notified when this item is ready to ship via email.

biological source

rabbit

Quality Level

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

Gene Information

human ... MT-ND1(4535)

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.

Specificity

The antibody detects endogenous levels of total MT-ND1 protein.

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.

Features and Benefits

Evaluate our antibodies with complete peace of mind. If the antibody does not perform in your application, we will issue a full credit or replacement antibody. Learn more.

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_germany

WGK 1

flash_point_f

Not applicable

flash_point_c

Not applicable


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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
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
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)
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

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