Conservation in subunit FV3 Back to subunit list
Human Complex I is shown with each of the 45 subunits colored a different color. The prosthetic groups, including FMN, FeS clusters, quinone, NADP+ and lipids are shown in space filling, colored by cpk.

Subunit FV3 is colored according to conservation. Magenta is most conserved, cyan is least conserved.

Isolated view of subunit FV3, residues 74-106 (chain K).
G103 in FV3    Undo

Mutations R26Q, K56N, E276K, and R200stop cannot be seen in this model. &
Atoms within 6 Å of subunit FV3 are shown in cpk according to the coloring scheme:

 FS1  nuoG  :M
 FV1  nuoF  :A
 FV2  nuoE  :O
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Protein wireframe   Protein cpk
Protein cartoon   Colored by sec. structure
Rainbow color: N-term-blue, C-term-red
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To show the atoms that contact residue 377 of NDUFV1 (:A),first select "color all" above, then type this: select within (6.0, 377:A); cpk only; display selected; center selected; zoom 1000; select 377:A; color white

There are no known clinical mutations in subunit FV3. It is the only subunit of human Complex I that has isoforms: 10kD (108 residues) and 50 kD (473 residues), which arise through alternative splicing. Both can be found in various tissues, and both allow assembly of Complex I. (References below)

Architecture of Human Mitochondrial Respiratory Megacomplex I2III2IV2.
Guo R, Zong S, Wu M, Gu J, Yang M.
Cell. 2017 Sep 7;170(6):1247-1257.e12. doi: 10.1016/j.cell.2017.07.050.(PubMed)

pdb ID (5xtd)

A novel isoform of the human mitochondrial complex I subunit NDUFV3. Dibley MG, Ryan MT, Stroud DA. FEBS Lett. 2017 Jan;591(1):109-117.

Identification and evolutionary analysis of tissue-specific isoforms of mitochondrial complex I subunit NDUFV3. Guerrero-Castillo S, Cabrera-Orefice A, Huynen MA, Arnold S. Biochim Biophys Acta Bioenerg. 2017 Mar;1858(3):208-217

Subunit NDUFV3 is present in two distinct isoforms in mammalian complex I. Bridges HR, Mohammed K, Harbour ME, Hirst J. Biochim Biophys Acta Bioenerg. 2017 Mar;1858(3):197-207.

This paper finds interactions between FV3 and other propteins by proteomics and cross-linking (in mouse).
The interactome of intact mitochondria by cross-linking mass spectrometry provides evidence for coexisting respiratory supercomplexes.
Liu F, Lössl P, Rabbitts BM, Balaban RS, Heck AJR.

Mol Cell Proteomics. 2018 Feb;17(2):216-232.