Protein Name: NADH-ubiquinone oxidoreductase 75 kDa subunit, mitochondrial
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UniprotKB/SwissProt ID: NDUS1_HUMAN (P28331)
Gene Name: NDUFS1
Organism: Homo sapiens (Human).
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 (By similarity). This is the largest subunit of complex I and it is a component of the iron-sulfur (IP) fragment of the enzyme. It may form part of the active site crevice where NADH is oxidized.
Other Modifications: View all modification sites in dbPTM
Protein Subcellular Localization: Mitochondrion inner membrane.
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Protein disease:
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| Disease database |
Database Entry |
Disease information | | KEGG | H00473 | Mitochondrial respiratory chain deficiencies (MRCD), including: Mitochondrial complex I deficiency ( | | OMIM | 157655 | NADH-UBIQUINONE OXIDOREDUCTASE Fe-S PROTEIN 1; NDUFS1 ;;COMPLEX I, MITOCHONDRIAL RESPIRATORY | | OMIM | 252010 | MITOCHONDRIAL COMPLEX I DEFICIENCY ;;NADH:Q(1) OXIDOREDUCTASE DEFICIENCY;; NADH-COENZYME Q R | | HPRD | 01145 | Mitochondrial complex I deficiency |
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Network with metabolic pathway:
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Graphical Visualization of S-nitrosylation Sites:
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| Overview of Protein S-nitrosylation Sites with Functional and Structural Information |  |
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