Dataset Information

The MICOS-MIB complex member PERM1 connects mitochondria with the sarcolemma via ankyrin B

ABSTRACT: Skeletal muscle subsarcolemmal mitochondria (SSM) and intermyofibrillar mitochondria subpopulations have distinct metabolic activity and sensitivity, though the mechanisms that localize SSM to peripheral areas of muscle fibers are poorly understood. A protein interaction study and complexome profiling identified PERM1 interacts with the MICOS-MIB complex. Ablation of Perm1 in mice reduced muscle force, decreased mitochondrial membrane potential and complex I activity, and reduced the numbers of SSM in skeletal muscle. We demonstrate PERM1 interacts with the intracellular adaptor protein ankyrin B (ANKB) that connects the cytoskeleton to the plasma membrane. In addition, we discovered a C-terminal transmembrane helix that anchors PERM1 into the outer mitochondrial membrane. We conclude PERM1 functions in the MICOS-MIB complex and acts as an adapter to connect the mitochondria with the sarcolemma via ANKB.

INSTRUMENT(S): Q Exactive

ORGANISM(S): Homo Sapiens (human) Mus Musculus (mouse)

TISSUE(S): Heart, Brain, Cell Culture, Muscle

SUBMITTER: Theresa Bock

LAB HEAD: Marcus Krüger

PROVIDER: PXD025745 | Pride | 2021-11-16

REPOSITORIES: Pride

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Dataset's files

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			Action	DRS
	20160226_AWH_ColID_103_Sriram_Krueger_Sol_KO_331m_12.raw	Raw
	20160226_AWH_ColID_103_Sriram_Krueger_Sol_KO_331m_13.raw	Raw
	20160226_AWH_ColID_103_Sriram_Krueger_Sol_KO_331m_14.raw	Raw
	20160226_AWH_ColID_103_Sriram_Krueger_Sol_KO_331m_15.raw	Raw
	20160226_AWH_ColID_103_Sriram_Krueger_Sol_KO_331m_16.raw	Raw

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Publications

PERM1 interacts with the MICOS-MIB complex to connect the mitochondria and sarcolemma via ankyrin B.

Bock Theresa T Türk Clara C Aravamudhan Sriram S Keufgens Lena L Bloch Wilhelm W Rozsivalova Dieu Hien DH Romanello Vanina V Nogara Leonardo L Blaauw Bert B Trifunovic Aleksandra A Braun Thomas T Krüger Marcus M

Nature communications 20210812 1

Skeletal muscle subsarcolemmal mitochondria (SSM) and intermyofibrillar mitochondria subpopulations have distinct metabolic activity and sensitivity, though the mechanisms that localize SSM to peripheral areas of muscle fibers are poorly understood. A protein interaction study and complexome profiling identifies PERM1 interacts with the MICOS-MIB complex. Ablation of Perm1 in mice reduces muscle force, decreases mitochondrial membrane potential and complex I activity, and reduces the numbers of ...[more]

PMID: 34385433

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