Name: U-Blot® ND1 Rabbit mAb
SKU: WR5189
Price: 268 USD
Availability: InStock

Product details

Background:catalytic activity:NADH + ubiquinone = NAD(+) + ubiquinol.,disease:Defects in MT-ND1 are a cause of Leber hereditary optic neuropathy (LHON) [MIM:535000]. LHON is a maternally inherited disease resulting in acute or subacute loss of central vision, due to optic nerve dysfunction. Cardiac conduction defects and neurological defects have also been described in some patients. LHON results from primary mitochondrial DNA mutations affecting the respiratory chain complexes.,disease:Defects in MT-ND1 are a cause of mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes syndrome (MELAS) [MIM:540000]. MELAS is a genetically heterogenious disorder, characterized by episodic vomiting, seizures, and recurrent cerebral insults resembling strokes and causing hemiparesis, hemianopsia, or cortical blindness.,disease:Defects in MT-ND1 may be associated with mitochondrial susceptibility to Alzheimer disease (AD) [MIM:502500].,disease:Defects in MT-ND1 may be associated with non-insulin-dependent diabetes mellitus (NIDDM).,function:Core subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I) that is believed to belong to the minimal assembly required for 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.,similarity:Belongs to the complex I subunit 1 family.,

Specifications

Size	50μL/100μL
Target	ND1
Reactivity	Human, Mouse, Rat
Applications	WB, IHC, IF, ELISA
MW(Calculated)	36kD
MW(Observed)	43kD
Host Species	Rabbit
Isotype	IgG,Kappa
Conjugate/Modification	Unmodified
Recommended Dilution Ratio	IHC 1:100-1:200; WB 1:2000-1:10000; IF 1:200-1:1000; ELISA 1:5000-1:20000; / Note: For IHC, we suggest antigen retrieval with TE buffer pH 9.0 (Cat#WT1004)
Formulation	PBS, 50% glycerol, 0.05% Proclin 300, 0.05%BSA
Purification	Recombinant Antibody expressed in animal component-free (ACF) media, purified via Protein A affinity chromatography.
Storage	-15°C to -25°C/1 year(Do not lower than -25°C)
Concentration
Clonality	Monoclonal
Clone Number	RM1423R
Immunogen	The specific immunogen used to produce this antibody is proprietary information.
Specificity	Endogenous
Gene Name	MT-ND1
Protein Name	NADH-ubiquinone oxidoreductase chain 1
Other Name	MT-ND1; / MTND1; / NADH1; / ND1; / NADH-ubiquinone oxidoreductase chain 1; / NADH dehydrogenase subunit 1
Species	Human
Gene ID	4535
Uniprot	P03886,
Species.1	Mouse
Gene ID.1
Uniprot.1	P03888
Species.2
Gene ID.2
Uniprot.2
Cellular Localization	Mitochondrion inner membrane; Multi-pass membrane protein .
Tissue Expression	Blood,Bone fossil,Bones,Breast cancer,Distant normal tissue,Glioma,
Signaling_pathway	Human Diseases >> Neurodegenerative disease >> Alzheimer disease;Human Diseases >> Neurodegenerative disease >> Parkinson disease;Human Diseases >> Neurodegenerative disease >> Amyotrophic lateral sclerosis;Human Diseases >> Neurodegenerative disease >> Huntington disease;Human Diseases >> Neurodegenerative disease >> Prion disease;Human Diseases >> Neurodegenerative disease >> Pathways of neurodegeneration - multiple diseases
Research Areas	>>Oxidative phosphorylation; / >>Metabolic pathways; / >>Thermogenesis; / >>Retrograde endocannabinoid signaling; / >>Alzheimer disease; / >>Parkinson disease; / >>Amyotrophic lateral sclerosis; / >>Huntington disease; / >>Prion disease; / >>Pathways of neurodegeneration - multiple diseases; / >>Chemical carcinogenesis - reactive oxygen species; / >>Diabetic cardiomyopathy
Function	Catalytic activity:NADH + ubiquinone = NAD(+) + ubiquinol.,Disease:Defects in MT-ND1 are a cause of Leber hereditary optic neuropathy (LHON) [MIM:535000]. LHON is a maternally inherited disease resulting in acute or subacute loss of central vision, due to optic nerve dysfunction. Cardiac conduction defects and neurological defects have also been described in some patients. LHON results from primary mitochondrial DNA mutations affecting the respiratory chain complexes.,Disease:Defects in MT-ND1 are a cause of mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes syndrome (MELAS) [MIM:540000]. MELAS is a genetically heterogenious disorder, characterized by episodic vomiting, seizures, and recurrent cerebral insults resembling strokes and causing hemiparesis, hemianopsia, or cortical blindness.,Disease:Defects in MT-ND1 may be associated with mitochondrial susceptibility to Alzheimer disease (AD) [MIM:502500].,Disease:Defects in MT-ND1 may be associated with non-insulin-dependent diabetes mellitus (NIDDM).,Function:Core subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I) that is believed to belong to the minimal assembly required for 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.,similarity:Belongs to the complex I subunit 1 family.,