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Protein Page:
MT-ND2 (human)

Overview
MT-ND2 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. Defects in MT-ND2 are a cause of Leber hereditary optic neuropathy (LHON). 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. Defects in MT-ND2 may be associated with susceptibility to Alzheimer disease mitochondrial (AD-MT). Alzheimer disease is a neurodegenerative disorder characterized by progressive dementia, loss of cognitive abilities, and deposition of fibrillar amyloid proteins as intraneuronal neurofibrillary tangles, extracellular amyloid plaques and vascular amyloid deposits. The major constituent of these plaques is the neurotoxic amyloid-beta-APP 40-42 peptide (s), derived proteolytically from the transmembrane precursor protein APP by sequential secretase processing. The cytotoxic C-terminal fragments (CTFs) and the caspase-cleaved products such as C31 derived from APP, are also implicated in neuronal death. Belongs to the complex I subunit 2 family. Note: This description may include information from UniProtKB.
Protein type: Membrane protein, multi-pass; EC 1.6.5.3; Membrane protein, integral; Oxidoreductase; Mitochondrial; Energy Metabolism - oxidative phosphorylation
Reference #:  P03891 (UniProtKB)
Alt. Names/Synonyms: mitochondrially encoded NADH dehydrogenase 2; MT-ND2; MTND2; NADH dehydrogenase subunit 2; NADH-ubiquinone oxidoreductase chain 2; NADH2; ND2; NU2M
Gene Symbols: MT-ND2
Molecular weight: 38,961 Da
Basal Isoelectric point: 9.84  Predict pI for various phosphorylation states
Select Structure to View Below

MT-ND2

Protein Structure Not Found.


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Modification Sites and Domains  

Modification Sites in Parent Protein, Orthologs, and Isoforms  
 

Show Multiple Sequence Alignment


 SS 

SS: The number of records in which this modification site was determined using site-specific methods. SS methods include amino acid sequencing, site-directed mutagenesis, modification site-specific antibodies, specific MS strategies, etc.


 MS 

MS: The number of records in which this modification site was assigned using ONLY proteomic discovery-mode mass spectrometry.


       human

 
0 1 T258-p LGGLPPLtGFLPKWA
0 1 Y298-p YFYLRLIySTSITLL
  mouse

 
T258 LGGLPPLTGFLPKWI
Y298 FFYIRLIYSTSLTMF
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