. Author manuscript; available in PMC: 2017 May 24.

Published in final edited form as: Nature. 2016 Nov 9;539(7630):575–578. doi: 10.1038/nature20170

Extended Data Table 1.

Fat1 interactors identified by TAP–MS that localize to the inner mitochondrial membrane

Gene	Protein	Function	Published Interactors	Ref.^†
NDUFS3	NADH:ubiquinone oxidoreductase core subunit S3	Core subunit of CI that belongs to the minimal assembly required for catalysis.	A number of CI, II, III, and V components	41,42
PHB	Prohibitin	Associates with and stabilizes respiratory complexes, particularly CI. Important for mitochondrial morphology and function, and growth control.	PHB2^, AFG3L2^, TIMM50^, ATAD3A^, UQCRC2^, STOML2^, and many respiratory complex subunits	⁴³^,⁴⁴ ⁴⁵
PHB2	Prohibitin 2	See above	See above	⁴³^,⁴⁴ 45
UQCRC2	Ubiquinol-cytochrome c reductase core protein II	Core subunit of CIII, required for its assembly.	NUDFS3^* (and other CI subunits), ATP5O^* (and other CV subunits), ATP5A1^, PHB^, AFG3L2^, ATAD3A^, HSPD1^*	44,46
ATP5F1	ATP synthase, H+ transporting, mitochondrial F0 complex subunit B1	Component of CV. ATP synthesis.	ATP5O^, ATP5A1^, SLC25A3^, STOML2^, HSPD1^, AFG3L2^ and many CI, CII, CIII, and CV subunits	⁴⁴^,⁴⁶ ⁴⁷
ATP5O	ATP synthase, H+ transporting, mitochondrial F1 complex, O subunit	See above.	ATP5F1^, ATP5A1^, UQCRC2^*, and other respiratory subunits	44,46
ATP5A1	ATP synthase, H+ transporting, mitochondrial F1 complex, α subunit 1, cardiac muscle	See above.	ATP5F1^, ATP5O^, UQCRC2^, AFG3L2^, STOML2^*, and other respiratory subunits	44,46
AFG3L2	AFG3 like matrix AAA peptidase subunit 2	AAA protease involved in IMM proteolysis. Forms a supercomplex with PHB at the IMM. PHB inhibits AFG3L2.	PHB^, ATP5F1^, ATP5A1^, UQCRC2^, SLC25A3^, TIMMDC1^ and many CI subunits	⁴⁴^,⁴⁶ ⁴⁸
ATAD3A	ATPase family, AAA domain containing 3A	Located at contact sites between OMM and IMM. Required for mitochondrial network organization, metabolism, and protein synthesis. Required for nucleoid stability and cell growth. Involved in complex I assembly.	PHB^, UQCRC2^, SLC25A3^, HSPD1^, and other CI and CV subunits.	⁴⁴^,⁴⁶ ⁴⁹^,⁵⁰ ⁵¹^,⁵²
STOML2	Stomatin like 2	Formation of cardiolipin-enriched microdomains. Recruits PHB to these microdomains to promote PHB function: assembly and stability of respiratory complexes. Promotes supercomplex formation, oxphos, CI and CII activities, and ATP production. Enhances T-cell proliferation.	PHB^, ATP5A1^, ATP5F1^, SLC25A3^, and other CIII and CV subunits	⁴⁴^,⁴⁶ ⁵³^,⁵⁴
TIMMDC1	Translocase of inner mitochondrial membrane domain containing 1	CI assembly: associates with core subunits of CI and components of the CI assembly machinery. Loss of function interrupts CI assembly, supercomplex formation, and mitochondrial respiration.	AFG3L2^*, core subunits of CI, CI assembly factors, CIII and CIV subunits	48
TIMM50	Translocase of inner mitochondrial membrane 50	Component of the TIM23 complex; mediates the translocation of transit peptide-containing proteins across the IMM.	PHB^*, and some CII subunits	46,55
ABCE1	ATP binding cassette subfamily E member 1	Inhibitor of RNA suppression. Regulation of mRNA turnover. Interacts with various CI subunits and CI assembly factors.		48,56
HSPA9	Heat shock protein family A (Hsp 70) member 9	Associates with cristae organizing system complex (MICOS), and modulates mito morphology. In yeast: binds to IMM to regulate mitochondrial import; optimizes translocation acting as an m-AAA protease to degrade/refold misfolded proteins. Implicated in cell proliferation, aging, cell fate determination, differentiation, tumor development.	DNAJA1^, DNAJA2^, DNAJA3^, HSPD1^, CI and CII subunits	⁴⁴^,⁴⁶ ⁵⁷^,⁵⁸ ⁵⁹^,⁶⁰
NIPSNAP1	Nipsnap homolog 1 (C. elegans)	Molecular function unknown.	Fat1^, HSPD1^	61
SLC25A1	Solute carrier family 25 member 1	Citrate/malate exchange. Important for the bioenergetics of hepatic cells; provides a carbon source for fatty acid and sterol biosynthesis, and NAD⁺ for the glycolytic pathway.		62
SLC25A3	Solute carrier family 25 member 3	Transport of phosphate groups (along with H+) from the cytosol to the mitochondrial matrix. Regulation of the mitochondrial permeability transition pore.	ATP5F1^, ATAD3A^, AFG3L2^, STOML2^, and many CI, II, III, and V subunits	44,46
SLC25A10	Solute carrier family 25 member 10	Translocation of malonate, malate and succinate in exchange for phosphate, sulfate, sulfite or thiosulfate across IMM.	CI subunits	46
SLC25A11	Solute carrier family 25 member 11	Transport of 2-oxoglutarate across the IMM in exchange for malate or other dicarboxylic acids; important for malate- aspartate shuttle, oxoglutarate/isocitrate shuttle, gluconeogenesis from lactate, and nitrogen metabolism.		63
SLC25A13	Solute carrier family 25 member 13	Calcium-dependent exchange of cytoplasmic glutamate with mitochondrial aspartate across the IMM.		64
SLC25A22	Solute carrier family 25 member 22	Transport of glutamate (with H+) across the IMM.	CII subunits	46,65
SLC25A33	Solute carrier family 25 member 33	Induced by IGF1/mTOR pathway. Controls mtDNA replication and transcription. Promotes mitochondrial respiration and cell growth, and protects from ROS-mediated differentiation during mitochondrial dysfunction.	HSPD1^*	44,66

Associated proteins that were also identified by the Fat1 TAP–MS screen.

^†

Refs ⁴¹^–⁶⁶ are cited in this table.