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ProteomicsMitochondrial depolarization stabilizes the vitamin B12 chaperone MMADHC in the cytosol to increase MTR activity
Of the ~1100 mitochondrial proteins, only a handful like PINK1 and ATFS-1 are known to stabilize and relocalize upon collapse of the proton motive force (PMF) to execute signaling roles. To systematically identify genes that increase exclusively at the protein level upon PMF collapse, we performed a...
ORGANISM(S): Homo sapiens (Human) 
2026-08-24 | PXD079746 | Pride
Proton motive force Mmadhc Vitamin b12 One carbon metabolism Metabolic compartmentalization 
GenomicsMitochondrial depolarization stabilizes the vitamin B12 chaperone MMADHC in the cytosol to increase MTR activity
Mitochondrial depolarization stabilizes the vitamin B12 chaperone MMADHC in the cytosol to increase MTR activity
PRJNA1478628 | ENA
TranscriptomicsMitochondrial depolarization stabilizes the vitamin B12 chaperone MMADHC in the cytosol to increase MTR activity
Of the ~1100 mitochondrial proteins, only a handful like PINK1 and ATFS-1 are known to stabilize and relocalize upon collapse of the proton motive force (PMF) to execute signaling roles. To systematically identify genes that increase exclusively at the protein level upon PMF collapse, we performed a...
ORGANISM(S): Homo sapiens 
2026-08-21 | GSE335487 | GEO
UnknownMMADHC premature termination codons in the pathogenesis of cobalamin D disorder: Potential of translational readthrough reconstitution.
Not available
S-EPMC7847965 | biostudies-literature
GenomicsMitochondrial protease Afg3l2 regulates the homeostasis of mouse hematopoietic stem and progenitor cells through Mmadhc [proteomics]
Mitochondrial protease Afg3l2 regulates the homeostasis of mouse hematopoietic stem and progenitor cells through Mmadhc [proteomics]
PRJNA1253635 | ENA
ProteomicsMitochondrial protease Afg3l2 regulates the homeostasis of mouse hematopoietic stem and progenitor cells through Mmadhc [proteomics]
In order to study how mitochondrial proteases function on mouse hematopoietic stem cells, the article constructed Afg3l2 knockout mice. When Afg3l2 was knocked out, mouse hematopoietic stem cells showed HSC depletion accompanied by amino acid accumulation and TCA overactivation. We performed transcr...
ORGANISM(S): Mus Mus musculus 
2025-12-15 | GSE295151 | GEO
UnknownMitochondrial depolarization stabilizes the vitamin B12 chaperone MMADHC in the cytosol to increase MTR activity.
Not available
S-EPMC12776266 | biostudies-literature
TranscriptomicsMitochondrial protease Afg3l2 regulates the homeostasis of mouse hematopoietic stem and progenitor cells through Mmadhc [RNA-Seq]
In order to study how mitochondrial proteases function on mouse hematopoietic stem cells, the article constructed Afg3l2 knockout mice. When Afg3l2 was knocked out, mouse hematopoietic stem cells showed HSC depletion accompanied by amino acid accumulation and TCA overactivation. We performed transcr...
ORGANISM(S): Mus musculus 
2025-12-15 | GSE295143 | GEO
GenomicsMitochondrial protease Afg3l2 regulates the homeostasis of mouse hematopoietic stem and progenitor cells through Mmadhc [RNA-Seq]
Mitochondrial protease Afg3l2 regulates the homeostasis of mouse hematopoietic stem and progenitor cells through Mmadhc [RNA-Seq]
PRJNA1253621 | ENA
UnknownReport of rapid diagnosis and precise management of MMADHC-related intracellular cobalamin defect.
Not available
S-EPMC8183291 | biostudies-literature
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