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3.D.1.6.1
The vertebrate H+-translocating NADH dehydrogenase (NDH) complex (45 subunits) (Cardol et al., 2004).  The 3-d structure of the 44 subunit complex (14 core subunits present in bacteria, plus 30 additional subunits) with a molecular weight of 1 MDa, has been solved at 5 Å resolution by single particle electron cryo-microscopy (Vinothkumar et al. 2014).The core subunits contain eight iron-sulphur clusters and 60 transmembrane helices. The structures of many of the supernumerary subunits were determined or modeled. The structure provided insight into the roles of the supernumerary subunits in regulation, assembly and homeostasis.  One such subunit, GRIM-19 or NDUFA13, (Q9P0J0 of the human homologue) is essential for membrane potential formation and NADH assembly (Lu and Cao 2008).  Stroud et al. 2016 showed that 25 of the 31 accessory subunits in the 45 subunit human NADH dehydrogenase complex are required for assembly of a functional complex, and 1 subunit is essential for cell viability. Quantitative proteomic analysis revealed that loss of each subunit affects the stability of other subunits residing in the same structural module (Stroud et al. 2016). Leber's hereditary optic neuropathy (LHON) in humans is associated with combinations of individually non-pathogenic missense mitochondrial DNA (mtDNA) variants, affecting the MT-ND4, MT-ND4L and MT-ND6 subunit genes of Complex I (Caporali et al. 2018). It has been proposed that the quinone reaction cavity is indeed from the matrix-side region covered by the ND3 TMH1-2 loop (Masuya et al. 2021). Resting mitochondrial complex I from Drosophila melanogaster adopts a helix-locked state (Padavannil et al. 2023).

Accession Number:P25708
Protein Name:51 (NuoF)
Length:464
Molecular Weight:50652.00
Species:Bos taurus (Bovine) [9913]
Location1 / Topology2 / Orientation3: Mitochondrion inner membrane1 / Peripheral membrane protein2 / Matrix side3
Substrate hydron

Cross database links:

RefSeq: NP_777233.1   
Entrez Gene ID: 287014   
Pfam: PF01512    PF10589    PF10531   
KEGG: bta:287014   

Gene Ontology

GO:0051539 F:4 iron, 4 sulfur cluster binding
GO:0010181 F:FMN binding
GO:0046872 F:metal ion binding
GO:0051287 F:NAD or NADH binding
GO:0008137 F:NADH dehydrogenase (ubiquinone) activity
GO:0022900 P:electron transport chain
GO:0006810 P:transport

References (2)

[1] “cDNA-derived amino acid sequence of the NADH-binding 51-kDa subunit of the bovine respiratory NADH dehydrogenase reveals striking similarities to a bacterial NAD(+)-reducing hydrogenase.”  Patel S.D.et.al.   2034666
[2] “Relationship between mitochondrial NADH-ubiquinone reductase and a bacterial NAD-reducing hydrogenase.”  Pilkington S.J.et.al.   1900194
Structure:
5lc5   5ldw   5LDX   5O31     

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FASTA formatted sequence
1:	MLAARRLLGG SLPARVSVRF SGDTTAPKKT SFGSLKDEDR IFTNLYGRHD WRLKGAQSRG 
61:	DWYKTKEILL KGPDWILGEV KTSGLRGRGG AGFPTGLKWS FMNKPSDGRP KYLVVNADEG 
121:	EPGTCKDREI IRHDPHKLVE GCLVGGRAMG ARAAYIYIRG EFYNEASNLQ VAIREAYEAG 
181:	LIGKNACGSG YDFDVFVVRG AGAYICGEET ALIESIEGKQ GKPRLKPPFP ADVGVFGCPT 
241:	TVANVETVAV SPTICRRGGA WFASFGRERN SGTKLFNISG HVNNPCTVEE EMSVPLKELI 
301:	EKHAGGVTGG WDNLLAVIPG GSSTPLIPKS VCETVLMDFD ALIQAQTGLG TAAVIVMDRS 
361:	TDIVKAIARL IEFYKHESCG QCTPCREGVD WMNKVMARFV RGDARPAEID SLWEISKQIE 
421:	GHTICALGDG AAWPVQGLIR HFRPELEERM QQFAQQHQAR QAAF