======= LONP1 =======


== Gene Information ==
  * **<color #00a2e8>Official Symbol</color>**: LONP1
  * **<color #00a2e8>Official Name</color>**: lon peptidase 1, mitochondrial
  * **<color #00a2e8>Aliases and Previous Symbols</color>**: N/A
  * **<color #00a2e8>Entrez ID</color>**: [[https://www.ncbi.nlm.nih.gov/gene/?term=9361|9361]]
  * **<color #00a2e8>UniProt</color>**: [[https://www.uniprot.org/uniprot/P36776|P36776]]
  * **<color #00a2e8>Interactions</color>**: [[https://thebiogrid.org/search.php?search=LONP1&organism=9606|BioGRID]]
  * **<color #00a2e8>PubMed articles</color>**: [[https://www.ncbi.nlm.nih.gov/pubmed/?term=gene%20LONP1|Open PubMed]]
  * **<color #00a2e8>OMIM</color>**: [[https://omim.org/entry/605490|Open OMIM]]

== Function Summary ==
  * **<color #00a2e8>Entrez Summary</color>**:  This gene encodes a mitochondrial matrix protein that belongs to the Lon family of ATP-dependent proteases. This protein mediates the selective degradation of misfolded, unassembled or oxidatively damaged polypeptides in the mitochondrial matrix. It may also have a chaperone function in the assembly of inner membrane protein complexes, and participate in the regulation of mitochondrial gene expression and maintenance of the integrity of the mitochondrial genome. Decreased expression of this gene has been noted in a patient with hereditary spastic paraplegia (PMID:18378094). Alternatively spliced transcript variants have been found for this gene. [provided by RefSeq, Feb 2013]. 
  * **<color #00a2e8>UniProt Summary</color>**:  ATP-dependent serine protease that mediates the selective degradation of misfolded, unassembled or oxidatively damaged polypeptides as well as certain short-lived regulatory proteins in the mitochondrial matrix. May also have a chaperone function in the assembly of inner membrane protein complexes. Participates in the regulation of mitochondrial gene expression and in the maintenance of the integrity of the mitochondrial genome. Binds to mitochondrial promoters and RNA in a single- stranded, site-specific, and strand-specific manner. May regulate mitochondrial DNA replication and/or gene expression using site- specific, single-stranded DNA binding to target the degradation of regulatory proteins binding to adjacent sites in mitochondrial promoters (PubMed:12198491, PubMed:15870080, PubMed:17420247, PubMed:8248235). Endogenous substrates include mitochondrial steroidogenic acute regulatory (StAR) protein, helicase Twinkle (TWNK) and the large ribosomal subunit protein bL32m. bL32m is protected from degradation by LONP1 when it is bound to a nucleic acid (RNA), but TWNK is not (PubMed:17579211, PubMed:28377575). {ECO:0000255|HAMAP-Rule:MF_03120, ECO:0000269|PubMed:12198491, ECO:0000269|PubMed:15870080, ECO:0000269|PubMed:17420247, ECO:0000269|PubMed:17579211, ECO:0000269|PubMed:28377575, ECO:0000269|PubMed:8248235}.

<button type='primary' size='sm' modal='Pfam_Domains'>Pfam Domains</button>
<button type='primary' size='sm' modal='GO_terms'>GO Terms</button>


<modal id='Pfam_Domains' size='lg' title='Pfam Domains'>
|AAA 2|
|Lon C|
|LON|
|AAA PrkA|
|AAA|
|AAA 5|
</modal>

<modal id='GO_terms' size='lg' title='GO Terms'>
|oxidation-dependent protein catabolic process|
|mitochondrial promoter sequence-specific DNA binding|
|response to aluminum ion|
|ATP-dependent peptidase activity|
|G-quadruplex DNA binding|
|mitochondrial DNA metabolic process|
|chaperone-mediated protein complex assembly|
|DNA polymerase binding|
|mitochondrial genome maintenance|
|protein quality control for misfolded or incompletely synthesized proteins|
|ADP binding|
|mitochondrial nucleoid|
|single-stranded RNA binding|
|single-stranded DNA binding|
|serine-type endopeptidase activity|
|cellular response to oxidative stress|
|aging|
|protein homooligomerization|
|response to hypoxia|
|response to decreased oxygen levels|
|mitochondrial matrix|
|response to metal ion|
|response to oxygen levels|
|response to oxidative stress|
|sequence-specific DNA binding|
|mitochondrion organization|
|protein complex oligomerization|
|modification-dependent protein catabolic process|
|response to inorganic substance|
|modification-dependent macromolecule catabolic process|
|proteolysis involved in cellular protein catabolic process|
|cellular protein catabolic process|
|protein catabolic process|
|DNA metabolic process|
|cellular protein-containing complex assembly|
|cellular macromolecule catabolic process|
|response to hormone|
|macromolecule catabolic process|
|organonitrogen compound catabolic process|
|response to abiotic stimulus|
|mitochondrion|
|proteolysis|
|response to endogenous stimulus|
|ATP binding|
|protein-containing complex assembly|
|cellular response to stress|
|organic substance catabolic process|
|cellular catabolic process|
|protein-containing complex subunit organization|
|membrane|
</modal>
\\

 === CRISPR Data ===
<button type='primary' size='small' modal='Compound_Hit'>Compound Hit</button>
<button type='primary' size='small' modal='Most_Correlated_Genes'>Most Correlated Genes in Chemogenomics</button>
<button type='primary' size='small' modal='Essential_Avana'>Tissues where Essential in the Avana Dataset (DepMap 20Q1)</button>

<modal id='Compound_Hit' size='lg' title='Compound Hit'>
^Screen^Score^
|[[:results:exp363|GSK-J4 1-1.25μM to day4 R07 exp363]]|-1.77|
|[[:results:exp185|L-BMAA 500 to 750μM on day4 R04 exp185]]|1.81|
|[[:results:exp96|BI-2536 0.02μM R03 exp96]]|1.85|
|[[:results:exp466|Cannabidiol 20μM R08 exp466]]|2.02|
</modal>

<modal id='Most_Correlated_Genes' size='lg' title='Most Correlated Genes in Chemogenomics'>
^Gene^Correlation^
|[[:human genes:s:slc25a26|SLC25A26]]|0.492|
|[[:human genes:a:aasdhppt|AASDHPPT]]|0.475|
|[[:human genes:t:triap1|TRIAP1]]|0.443|
|[[:human genes:r:romo1|ROMO1]]|0.436|
|[[:human genes:m:mrpl23|MRPL23]]|0.421|
|[[:human genes:t:taz|TAZ]]|0.418|
|[[:human genes:o:opa1|OPA1]]|0.416|
</modal>

<modal id='Essential_Avana' size='lg' title='Tissues where Essential in the Avana Dataset (DepMap 20Q1)'>
Global Fraction of Cell Lines Where Essential: 709/739
^Tissue^Fraction Of Cell Lines Where Essential^
|1290807.0|1/1|
|909776.0|1/1|
|bile duct|27/28|
|blood|27/28|
|bone|26/26|
|breast|32/33|
|central nervous system|56/56|
|cervix|4/4|
|colorectal|17/17|
|esophagus|13/13|
|fibroblast|1/1|
|gastric|16/16|
|kidney|18/21|
|liver|18/20|
|lung|72/75|
|lymphocyte|16/16|
|ovary|25/26|
|pancreas|23/24|
|peripheral nervous system|15/16|
|plasma cell|13/15|
|prostate|0/1|
|skin|23/24|
|soft tissue|9/9|
|thyroid|2/2|
|upper aerodigestive|21/22|
|urinary tract|28/29|
|uterus|4/5|
</modal>

== Essentiality in NALM6  ==
    * **<color #00a2e8>Essentiality Rank</color>**: 926
    * **<color #00a2e8>Expression level (log2 read counts)</color>**: 7.51 
<button type='primary' size='small' modal='Dist_expr'>Expression Distribution</button>
<modal id='Dist_expr' size='lg' title='LONP1 Expression in NALM6 Cells: 7.51'>
{{:chemogenomics:nalm6 dist.png?nolink |}}
</modal>