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Details on Person UniProt:Q96TA2 YME1L1

Class:IdReferenceGeneProduct:156088
_chainChangeLogchain:1-773 added on Fri February 6 2015
_displayNameUniProt:Q96TA2 YME1L1
_timestamp2025-02-21 19:45:01
chainchain:1-773
checksumFB77990F4D7B3A58
commentFUNCTION ATP-dependent metalloprotease that catalyzes the degradation of folded and unfolded proteins with a suitable degron sequence in the mitochondrial intermembrane region (PubMed:24315374, PubMed:26923599, PubMed:27786171, PubMed:31695197, PubMed:33237841, PubMed:36206740). Plays an important role in regulating mitochondrial morphology and function by cleaving OPA1 at position S2, giving rise to a form of OPA1 that promotes maintenance of normal mitochondrial structure and mitochondrial protein metabolism (PubMed:18076378, PubMed:26923599, PubMed:27495975, PubMed:33237841). Ensures cell proliferation, maintains normal cristae morphology and complex I respiration activity, promotes antiapoptotic activity and protects mitochondria from the accumulation of oxidatively damaged membrane proteins (PubMed:22262461). Required to control the accumulation of nonassembled respiratory chain subunits (NDUFB6, OX4 and ND1) (PubMed:22262461). Involved in the mitochondrial adaptation in response to various signals, such as stress or developmental cues, by mediating degradation of mitochondrial proteins to rewire the mitochondrial proteome (PubMed:31695197). Catalyzes degradation of mitochondrial proteins, such as translocases, lipid transfer proteins and metabolic enzymes in response to nutrient starvation in order to limit mitochondrial biogenesis: mechanistically, YME1L is activated by decreased phosphatidylethanolamine levels caused by LPIN1 activity in response to mTORC1 inhibition (PubMed:31695197). Acts as a regulator of adult neural stem cell self-renewal by promoting mitochondrial proteome rewiring, preserving neural stem and progenitor cells self-renewal (By similarity). Required for normal, constitutive degradation of PRELID1 (PubMed:27495975). Catalyzes the degradation of OMA1 in response to membrane depolarization (PubMed:26923599). Mediates degradation of TIMM17A downstream of the integrated stress response (ISR) (PubMed:24315374). Catalyzes degradation of MICU1 when MICU1 is not assembled via an interchain disulfide (PubMed:36206740).CATALYTIC ACTIVITY ATP + H2O = ADP + phosphate + H(+)COFACTOR Binds 1 zinc ion per subunit.BIOPHYSICOCHEMICAL PROPERTIES Homohexamer; may also form heterohexamers (PubMed:27786171). Exists in several complexes of 600-1100 kDa (PubMed:22262461, PubMed:27495975). Interacts with AFG1L (PubMed:26759378).SUBCELLULAR LOCATION High expression in cardiac and skeletal muscle mitochondria.PTM Proteolytically processed by mitochondrial processing peptidase (MPP) to generate the mature form (PubMed:27495975). Degraded in an OMA1-dependent manner in response to oxidative stress (PubMed:27495975).DISEASE The disease may be caused by variants affecting the gene represented in this entry.MISCELLANEOUS Mutagenesis of Glu-543 to Gln does not complement excessive accumulation of subunits (NDUFB6, COX4,ND1) due to YME1 deletion mutant. Probably has no ATPase activity.SIMILARITY In the N-terminal section; belongs to the AAA ATPase family.SIMILARITY In the C-terminal section; belongs to the peptidase M41 family.
created[InstanceEdit:143527] Schmidt, EE, 2004-11-12 07:45:10
descriptionrecommendedName: ATP-dependent zinc metalloprotease YME1L1 ecNumber evidence="7 9 10 11 12 13"3.4.24.- ecNumber evidence="11"3.6.-.- alternativeName: ATP-dependent metalloprotease FtsH1 alternativeName: Meg-4 alternativeName: Presenilin-associated metalloprotease shortName: PAMP alternativeName: YME1-like protein 1
geneNameYME1L1
FTSH1
YME1L
UNQ1868/PRO4304
identifierQ96TA2
isSequenceChangedFALSE
keywordAlternative splicing
ATP-binding
Disease variant
Hydrolase
Membrane
Metal-binding
Metalloprotease
Mitochondrion
Mitochondrion inner membrane
Nucleotide-binding
Protease
Proteomics identification
Reference proteome
Transmembrane
Transmembrane helix
Zinc
modified[InstanceEdit:9836292] Weiser, Joel, 2023-05-25
[InstanceEdit:9852000] Weiser, Joel, 2023-11-03
[InstanceEdit:9917590] Weiser, Joel, 2024-08-09
[InstanceEdit:9926675] Weiser, Joel, 2024-11-03
[InstanceEdit:9939033] Weiser, Joel, 2025-02-21
nameYME1L1
referenceDatabase[ReferenceDatabase:2] UniProt
referenceGene[ReferenceDNASequence:8998102] ENSEMBL:ENSG00000136758 YME1L1 [Homo sapiens]
secondaryIdentifierYMEL1_HUMAN
B4DNM1
D3DRV8
D3DRV9
Q5T8D9
Q9H1Q0
Q9UMR9
sequenceLength773
species[Species:48887] Homo sapiens
(isoformParent)[ReferenceIsoform:156089] UniProt:Q96TA2-2 YME1L1 [Homo sapiens]
[ReferenceIsoform:413126] UniProt:Q96TA2-1 YME1L1 [Homo sapiens]
[ReferenceIsoform:8974552] UniProt:Q96TA2-3 YME1L1 [Homo sapiens]
(referenceEntity)[EntityWithAccessionedSequence:8949648] YME1L1 [mitochondrial inner membrane] [Homo sapiens]
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No pathways have been reviewed or authored by UniProt:Q96TA2 YME1L1 (156088)