Featured publications

Combinatorial sequence elements fine-tune mitochondrial protein import to facilitate dual localization

Yan Y.*, Wei, L.*, Schodl T., Kozul K.-L., Behrens M., Plassmeyer S.P., Holehouse A.S., and N.M. Niemi. 2026. bioRxiv. https://doi.org/10.64898/2026.07.31.742112

*Co-first authors

Sustained loss of Pptc7 triggers variable skeletal muscle dysfunction and diminished body mass through dysregulation of BNIP3

Lochetto T.M., Menezes T.N., Cho K., Vegesna S., Morhaus M.M., Ferey J.L.A., Forny M., Shriver L.P., Meyer G.A., Patti G.J., and N.M. Niemi. 2026. bioRxiv. https://doi.org/10.64898/2026.07.13.738264

Recessive PPTC7 deficiency triggers excessive mitophagy to cause a severe inborn error of metabolism with hypomyelinating leukodystrophy

Kozul, K.-L.* AlAsmari A.*, Alharby E., Zakzouk R.S., Yan Y., Mushiba A.M., Harrelson E.N., Ayach M., Cho K., Zahid H., de Luna Vitorino F.N., Searfoss R.M., Liu X., Saleh M.A., Mayo L., Wei L., Aldawood A., Alsuhaibani L., Bafail M.A., Menezes T.N., Samman M., Pletcher H., Sandokji I.A., Borhan W.M., Lochetto T.M., Alamri A., Mudayfin W., Syed M., Shriver L.P., Garcia B.A., Faqeih E.A., Patti G.J., Niemi N.M.^ and N.A.M. Almontashiri^. February 2026, PREPRINT (Version 1) Research Square [https://doi.org/10.21203/rs.3.rs-8815446/v1]Research Square 225(4):e202507116.

*Co-first authors, ^co-corresponding authors

Mitochondrial presequences harbor variable strengths to maintain organellar function

Yan Y., Erdenepurev B., Menezes, T.N., Collinson I., and N.M. Niemi. 2026. J Cell Biol. 225(4):e202507116.

See also: JCB Spotlight “How strong is a mitochondrial targeting signal?”

Coordinating BNIP3/NIX-mediated mitophagy in space and time

N.M. Niemi & J.R. Friedman. 2024. Biochem Soc Trans BST20221364.

Dual-localized PPTC7 limits mitophagy through proximal and dynamic interactions with BNIP3 and NIX

Wei L., Gok M.O., Svoboda J.D., Kozul K., Forny M., Friedman J.R., and N.M. Niemi. 2024. Life Sci. Alliance 2024 Jul 11; 7(9):e202402765.

PPTC7 maintains mitochondrial protein content by suppressing receptor-mediated mitophagy

Niemi, N.M.*, Serrano L.R., Muehlbauer L. K., Balnis C. E., Wei, L., Smith A.J., Kozul, K., Forny, M., Connor O.M., Rashan E.H., Shishkova E., Schueler K.L., Keller M.P., Attie A.D., Friedman, J.R., Pagan J., Coon J.J., and D.J. Pagliarini. 2023. Nat Commun. 14(1):6431.

Pptc7 is an essential phosphatase for promoting mitochondrial metabolism and biogenesis

Niemi, N.M., Wilson, G.M., Overmyer, K.A., Vogtle, F.N., Lohman D.C., Schueler, K.L., Attie, A.D., Meisinger C., Coon, J.J., and D.J. Pagliarini. 2019. Nat Commun. 10(1):3197. PMID: 31324765

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Integrative Proteomics and Biochemical Analyses Define Ptc6p as the Saccharomyces cerevisiae Pyruvate Dehydrogenase Phosphatase

Guo X., Niemi N.M., Coon J.J., and D.J. Pagliarini. 2017. J. Biol. Chem. 292(28):11751-11759. PMID: 28539364

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Ptc7p dephosphorylates select mitochondrial proteins to enhance metabolic function

Guo X.*, Niemi N.M.*, Hutchins, P.D., Condon, S.G.F., Jochem, A., Ulbrich, A., Higbee, A.J., Russell, J.D., Senes, A., Coon, J.J., and D.J. Pagliarini. 2017. Cell Rep. 18(2):307-13. *Co-first author. PMID: 28076776

Collaborative work

 

Basal BNIP3/NIX mitophagy is controlled by selective protection of sentinel receptors

Kumar A., Love A., Kozul K.-L., Gok M., Niemi N.M., and J.R. Friedman. 2026. bioRxiv.

doi: https://doi.org/10.64898/2026.06.25.734517

Alternative organelle targeting of OPA1 mediates fatty acid release from lipid droplets

Li X., Voronin D., Bhattacharyya R., Klein J., Haas M., Cho W.J., Robinson C.G., Throm R.E., Wu G., Li C., Sapkoa Y., Niemi N.M., Pruett-Miller S.M., Opferman J.T., and C.-L. Chang. Alternative organelle targeting of OPA1 mediates fatty acid release from lipid droplets. 2026. bioRxiv. https://doi.org/10.64898/2026.05.07.723579

The MitoLuc assay for the analysis of the mechanism of mitochondrial protein import

Needs H. I.*, Yan Y.*, Niemi N.M.^, and I. Collinson^. 2024. Methods Enzymol. Online ahead of print.

The pyruvate dehydrogenase complex regulates mitophagic selectivity independently of its catalytic activity

Kolitsida P., Nolic V., Zhou J., Niemi N.M., Dengjel J., and H. Abeliovich. 2023. Life Sci Alliance. 6(9):e202302149.

Enhanced immunoprecipitation techniques for the identification of RNA-binding protein partners: IGF2BP1 interactions in mammary epithelial cells

Fakhraldeen S.A., Berry S.M., Beebe D.J., Roopra A., Bisbach C.M., Spiegelman V.S., Niemi N.M. and C.M. Alexander. 2022. J. Biol. Chem. volume 298, issue 3, 101649.

Quantitative shotgun proteome analysis by direct infusion

Meyer J.G., Niemi N.M., Pagliarini D.J., and J.J.Coon. 2020. Nature Methods volume 17, pages1222–1228.

A survey-based analysis of the academic job market

Fernandes J.D., Sarabipour S., Smith C.T., Niemi N.M., Jadavji N.M., Kozik A.J., Holehouse A.S., Pejaver V., Symmons O., Bisson Filho A.W., and A. Haage. eLife 2020;9:e54097

DOI: 10.7554/eLife.54097.

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