Overview
| Uniprot ID | Q96TA2 |
|---|
| Protein Name | ATP-dependent zinc metalloprotease YME1L1 |
|---|
| Gene Name | YME1L1 |
|---|
| Organism | Homo sapiens |
|---|
Kla Sites from experimental identification
| Position |
Flanking peptide |
| 691 |
IAKRMVTKFGMSEKL |
Function
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, YME1L1 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). Cleaves STARD7 mitochondrial targeting sequence (PubMed:29301859)
Protein Sequence
10
MFSLSSTVQP
20
QVTVPLSHLI
30
NAFHTPKNTS
40
VSLSGVSVSQ
50
NQHRDVVPEH
60
EAPSSECMFS
70
DFLTKLNIVS
80
IGKGKIFEGY
90
RSMFMEPAKR
100
MKKSLDTTDN
110
WHIRPEPFSL
120
SIPPSLNLRD
130
LGLSELKIGQ
140
IDQLVENLLP
150
GFCKGKNISS
160
HWHTSHVSAQ
170
SFFENKYGNL
180
DIFSTLRSSC
190
LYRHHSRALQ
200
SICSDLQYWP
210
VFIQSRGFKT
220
LKSRTRRLQS
230
TSERLAETQN
240
IAPSFVKGFL
250
LRDRGSDVES
260
LDKLMKTKNI
270
PEAHQDAFKT
280
GFAEGFLKAQ
290
ALTQKTNDSL
300
RRTRLILFVL
310
LLFGIYGLLK
320
NPFLSVRFRT
330
TTGLDSAVDP
340
VQMKNVTFEH
350
VKGVEEAKQE
360
LQEVVEFLKN
370
PQKFTILGGK
380
LPKGILLVGP
390
PGTGKTLLAR
400
AVAGEADVPF
410
YYASGSEFDE
420
MFVGVGASRI
430
RNLFREAKAN
440
APCVIFIDEL
450
DSVGGKRIES
460
PMHPYSRQTI
470
NQLLAEMDGF
480
KPNEGVIIIG
490
ATNFPEALDN
500
ALIRPGRFDM
510
QVTVPRPDVK
520
GRTEILKWYL
530
NKIKFDQSVD
540
PEIIARGTVG
550
FSGAELENLV
560
NQAALKAAVD
570
GKEMVTMKEL
580
EFSKDKILMG
590
PERRSVEIDN
600
KNKTITAYHE
610
SGHAIIAYYT
620
KDAMPINKAT
630
IMPRGPTLGH
640
VSLLPENDRW
650
NETRAQLLAQ
660
MDVSMGGRVA
670
EELIFGTDHI
680
TTGASSDFDN
690
ATKIAKRMVT
700
KFGMSEKLGV
710
MTYSDTGKLS
720
PETQSAIEQE
730
IRILLRDSYE
740
RAKHILKTHA
750
KEHKNLAEAL
760
LTYETLDAKE
770
IQIVLEGKKL
EVR
Gene Ontology
| Classification |
GO ID |
Description |
| Cellular Component |
GO:0016020 |
membrane |
| Cellular Component |
GO:0005743 |
mitochondrial inner membrane |
| Cellular Component |
GO:0005739 |
mitochondrion |
| Molecular Function |
GO:0005524 |
ATP binding |
| Molecular Function |
GO:0016887 |
ATP hydrolysis activity |
| Molecular Function |
GO:0004176 |
ATP-dependent peptidase activity |
| Molecular Function |
GO:0046872 |
metal ion binding |
| Molecular Function |
GO:0004222 |
metalloendopeptidase activity |
| Biological Process |
GO:0008283 |
cell population proliferation |
| Biological Process |
GO:0009267 |
cellular response to starvation |
| Biological Process |
GO:0035694 |
mitochondrial protein catabolic process |
| Biological Process |
GO:0034982 |
mitochondrial protein processing |
| Biological Process |
GO:0007005 |
mitochondrion organization |
| Biological Process |
GO:0043066 |
negative regulation of apoptotic process |
| Biological Process |
GO:0097150 |
neuronal stem cell population maintenance |
| Biological Process |
GO:0010636 |
positive regulation of mitochondrial fusion |
| Biological Process |
GO:0034214 |
protein hexamerization |
| Biological Process |
GO:0006515 |
protein quality control for misfolded or incompletely synthesized proteins |
| Biological Process |
GO:2000035 |
regulation of stem cell division |
Reference
PMID: N/A