Q1L5Z9 (LONF2_HUMAN) Reviewed, UniProtKB/Swiss-Prot

Names and origin

Protein names

Recommended name:
LON peptidase N-terminal domain and RING finger protein 2

Alternative name(s):
Neuroblastoma apoptosis-related protease
RING finger protein 192

Gene names

Name:	LONRF2
Synonyms:	RNF192

Organism

Homo sapiens (Human) [Reference proteome]

Taxonomic identifier

9606 [NCBI]

Taxonomic lineage

cellular organisms › Eukaryota › Opisthokonta › Metazoa › Eumetazoa › Bilateria › Deuterostomia › Chordata › Craniata › Vertebrata › Gnathostomata › Teleostomi › Euteleostomi › Sarcopterygii › Tetrapoda › Amniota › Mammalia › Theria › Eutheria › Euarchontoglires › Primates › Haplorrhini › Simiiformes › Catarrhini › Hominoidea › Hominidae › Homininae › Homo

Protein attributes

Sequence length	754 AA.
Sequence status	Complete.
Protein existence	Evidence at transcript level

General annotation (Comments)

Sequence similarities

Contains 1 Lon domain. Contains 1 RING-type zinc finger. Contains 6 TPR repeats.

Ontologies

Keywords
Coding sequence diversity	Alternative splicing Polymorphism
Domain	Repeat TPR repeat Zinc-finger
Ligand	Metal-binding Zinc
Technical term	Complete proteome Reference proteome
Gene Ontology (GO)
Biological_process	proteolysis Inferred from electronic annotation. Source: InterPro
Molecular_function	ATP-dependent peptidase activity Inferred from electronic annotation. Source: InterPro zinc ion binding Inferred from electronic annotation. Source: InterPro
Complete GO annotation...

Alternative products

This entry describes 2 isoforms produced by alternative splicing. [Align] [Select]

Isoform 1 (identifier: Q1L5Z9-1)

This isoform has been chosen as the 'canonical' sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry.

Isoform 2 (identifier: Q1L5Z9-2)

The sequence of this isoform differs from the canonical sequence as follows:
     1-243: Missing.

Sequence annotation (Features)

	Feature key	Position(s)	Length	Description	Graphical view	Feature identifier
Molecule processing
	Chain	1 – 754	754	LON peptidase N-terminal domain and RING finger protein 2		PRO_0000277670
Regions
	Repeat	23 – 58	36	TPR 1
	Repeat	59 – 91	33	TPR 2
	Repeat	197 – 230	34	TPR 3
	Repeat	231 – 264	34	TPR 4
	Repeat	266 – 298	33	TPR 5
	Repeat	447 – 483	37	TPR 6
	Domain	530 – 738	209	Lon
	Zinc finger	449 – 487	39	RING-type
	Compositional bias	3 – 12	10	Poly-Pro
Natural variations
	Alternative sequence	1 – 243	243	Missing in isoform 2.		VSP_023079
	Natural variant	183	1	L → P. Corresponds to variant rs4851287 [ dbSNP | Ensembl ].		VAR_030590
	Natural variant	426	1	L → P. Ref.1 Ref.2 Ref.4 Corresponds to variant rs4851287 [ dbSNP | Ensembl ].		VAR_054639
	Natural variant	562	1	R → W in a colorectal cancer sample; somatic mutation. Ref.5		VAR_035953
Experimental info
	Sequence conflict	648	1	E → Q in AAY84832. Ref.1

Sequences

	Sequence		Length	Mass (Da)
	Isoform 1 [UniParc]. Last modified November 30, 2010. Version 3. Checksum: 688EE74DCF7893D3	FASTA	754	83,654
	10 20 30 40 50 60 MSPEPVPPPP PPQCPGCDRA EPIAQRLEEG DEAFRAGDYE MAAELFRSML AGLAQPDRGL 70 80 90 100 110 120 CLRLGDALAR AGRLPEALGA FRGAARLGAL RPEELEELAG GLVRAVGLRD RPLSAENPGG 130 140 150 160 170 180 EPEAPGEGGP APEPRAPRDL LGCPRCRRLL HKPVTLPCGL TVCKRCVEPG PARPQVRRVN 190 200 210 220 230 240 VVLSGLLEKC FPAECRLRRL AGQARSLQRQ QQPEAALLRC DQALELAPDD NSLLLLRAEL 250 260 270 280 290 300 YLTMKNYEQA LQDASAACQN EPLLIKGHQV KAQALSGLGR SKEVLKEFLY CLALNPECNS 310 320 330 340 350 360 VKKEAQKVMC EVLFSATANV HENLTSSIQS RLKAQGHSHM NAQALLEEGD AGSSENSSEK 370 380 390 400 410 420 SDMLGNTNSS VLYFILGLHF EEDKKALESI LPTAPSAGLK RQFPDDVEDA PDLNAPGKIP 430 440 450 460 470 480 KKDLSLQRSP NSETEESQGL SLDVTDFECA LCMRLLFEPV TTPCGHTFCL KCLERCLDHA 490 500 510 520 530 540 PHCPLCKDKL SELLASRNFN ITVLAEELIF RYLPDELSDR KRIYDEEMSE LSNLTRDVPI 550 560 570 580 590 600 FVCAMAFPTV PCPLHVFEPR YRLMIRRCME TGTKRFGMCL SAEHAGLSEY GCMLEIKDVR 610 620 630 640 650 660 TFPDGSSVVD AIGISRFRVL SHRHRDGYNT ADIEYLEDEK VEGPEYEELA ALHDSVHQQS 670 680 690 700 710 720 VSWFASLQDR MKEQILSHFG VMPDREPEPQ SNPSGPAWSW WILAVLPLER KAQLAILGMT 730 740 750 SLKERLLAIR RILVIITRKM NSRQELANAR ERNN
	Isoform 2 [UniParc]. Checksum: AE7D01250CE317F9	FASTA	511	57,487
	10 20 30 40 50 60 MKNYEQALQD ASAACQNEPL LIKGHQVKAQ ALSGLGRSKE VLKEFLYCLA LNPECNSVKK 70 80 90 100 110 120 EAQKVMCEVL FSATANVHEN LTSSIQSRLK AQGHSHMNAQ ALLEEGDAGS SENSSEKSDM 130 140 150 160 170 180 LGNTNSSVLY FILGLHFEED KKALESILPT APSAGLKRQF PDDVEDAPDL NAPGKIPKKD 190 200 210 220 230 240 LSLQRSPNSE TEESQGLSLD VTDFECALCM RLLFEPVTTP CGHTFCLKCL ERCLDHAPHC 250 260 270 280 290 300 PLCKDKLSEL LASRNFNITV LAEELIFRYL PDELSDRKRI YDEEMSELSN LTRDVPIFVC 310 320 330 340 350 360 AMAFPTVPCP LHVFEPRYRL MIRRCMETGT KRFGMCLSAE HAGLSEYGCM LEIKDVRTFP 370 380 390 400 410 420 DGSSVVDAIG ISRFRVLSHR HRDGYNTADI EYLEDEKVEG PEYEELAALH DSVHQQSVSW 430 440 450 460 470 480 FASLQDRMKE QILSHFGVMP DREPEPQSNP SGPAWSWWIL AVLPLERKAQ LAILGMTSLK 490 500 510 ERLLAIRRIL VIITRKMNSR QELANARERN N

References

	« Hide 'large scale' referencesShow 'large scale' references »
[1]	"Differential screening of novel gene in apoptotic neuronal cells and its functional study during embryonic development." Choi D.K. Submitted (APR-2005) to the EMBL/GenBank/DDBJ databases Cited for: NUCLEOTIDE SEQUENCE [MRNA] (ISOFORM 1), VARIANT PRO-426.
[2]	"Complete sequencing and characterization of 21,243 full-length human cDNAs." Ota T., Suzuki Y., Nishikawa T., Otsuki T., Sugiyama T., Irie R., Wakamatsu A., Hayashi K., Sato H., Nagai K., Kimura K., Makita H., Sekine M., Obayashi M., Nishi T., Shibahara T., Tanaka T., Ishii S. , Yamamoto J., Saito K., Kawai Y., Isono Y., Nakamura Y., Nagahari K., Murakami K., Yasuda T., Iwayanagi T., Wagatsuma M., Shiratori A., Sudo H., Hosoiri T., Kaku Y., Kodaira H., Kondo H., Sugawara M., Takahashi M., Kanda K., Yokoi T., Furuya T., Kikkawa E., Omura Y., Abe K., Kamihara K., Katsuta N., Sato K., Tanikawa M., Yamazaki M., Ninomiya K., Ishibashi T., Yamashita H., Murakawa K., Fujimori K., Tanai H., Kimata M., Watanabe M., Hiraoka S., Chiba Y., Ishida S., Ono Y., Takiguchi S., Watanabe S., Yosida M., Hotuta T., Kusano J., Kanehori K., Takahashi-Fujii A., Hara H., Tanase T.-O., Nomura Y., Togiya S., Komai F., Hara R., Takeuchi K., Arita M., Imose N., Musashino K., Yuuki H., Oshima A., Sasaki N., Aotsuka S., Yoshikawa Y., Matsunawa H., Ichihara T., Shiohata N., Sano S., Moriya S., Momiyama H., Satoh N., Takami S., Terashima Y., Suzuki O., Nakagawa S., Senoh A., Mizoguchi H., Goto Y., Shimizu F., Wakebe H., Hishigaki H., Watanabe T., Sugiyama A., Takemoto M., Kawakami B., Yamazaki M., Watanabe K., Kumagai A., Itakura S., Fukuzumi Y., Fujimori Y., Komiyama M., Tashiro H., Tanigami A., Fujiwara T., Ono T., Yamada K., Fujii Y., Ozaki K., Hirao M., Ohmori Y., Kawabata A., Hikiji T., Kobatake N., Inagaki H., Ikema Y., Okamoto S., Okitani R., Kawakami T., Noguchi S., Itoh T., Shigeta K., Senba T., Matsumura K., Nakajima Y., Mizuno T., Morinaga M., Sasaki M., Togashi T., Oyama M., Hata H., Watanabe M., Komatsu T., Mizushima-Sugano J., Satoh T., Shirai Y., Takahashi Y., Nakagawa K., Okumura K., Nagase T., Nomura N., Kikuchi H., Masuho Y., Yamashita R., Nakai K., Yada T., Nakamura Y., Ohara O., Isogai T., Sugano S. Nat. Genet. 36:40-45(2004) [PubMed] [Europe PMC] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORM 2), VARIANT PRO-426. Tissue: Hippocampus.
[3]	"Generation and annotation of the DNA sequences of human chromosomes 2 and 4." Hillier L.W., Graves T.A., Fulton R.S., Fulton L.A., Pepin K.H., Minx P., Wagner-McPherson C., Layman D., Wylie K., Sekhon M., Becker M.C., Fewell G.A., Delehaunty K.D., Miner T.L., Nash W.E., Kremitzki C., Oddy L., Du H. , Sun H., Bradshaw-Cordum H., Ali J., Carter J., Cordes M., Harris A., Isak A., van Brunt A., Nguyen C., Du F., Courtney L., Kalicki J., Ozersky P., Abbott S., Armstrong J., Belter E.A., Caruso L., Cedroni M., Cotton M., Davidson T., Desai A., Elliott G., Erb T., Fronick C., Gaige T., Haakenson W., Haglund K., Holmes A., Harkins R., Kim K., Kruchowski S.S., Strong C.M., Grewal N., Goyea E., Hou S., Levy A., Martinka S., Mead K., McLellan M.D., Meyer R., Randall-Maher J., Tomlinson C., Dauphin-Kohlberg S., Kozlowicz-Reilly A., Shah N., Swearengen-Shahid S., Snider J., Strong J.T., Thompson J., Yoakum M., Leonard S., Pearman C., Trani L., Radionenko M., Waligorski J.E., Wang C., Rock S.M., Tin-Wollam A.-M., Maupin R., Latreille P., Wendl M.C., Yang S.-P., Pohl C., Wallis J.W., Spieth J., Bieri T.A., Berkowicz N., Nelson J.O., Osborne J., Ding L., Meyer R., Sabo A., Shotland Y., Sinha P., Wohldmann P.E., Cook L.L., Hickenbotham M.T., Eldred J., Williams D., Jones T.A., She X., Ciccarelli F.D., Izaurralde E., Taylor J., Schmutz J., Myers R.M., Cox D.R., Huang X., McPherson J.D., Mardis E.R., Clifton S.W., Warren W.C., Chinwalla A.T., Eddy S.R., Marra M.A., Ovcharenko I., Furey T.S., Miller W., Eichler E.E., Bork P., Suyama M., Torrents D., Waterston R.H., Wilson R.K. Nature 434:724-731(2005) [PubMed] [Europe PMC] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
[4]	"The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)." The MGC Project Team Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORM 2), VARIANT PRO-426. Tissue: Brain.
[5]	"The consensus coding sequences of human breast and colorectal cancers." Sjoeblom T., Jones S., Wood L.D., Parsons D.W., Lin J., Barber T.D., Mandelker D., Leary R.J., Ptak J., Silliman N., Szabo S., Buckhaults P., Farrell C., Meeh P., Markowitz S.D., Willis J., Dawson D., Willson J.K.V. , Gazdar A.F., Hartigan J., Wu L., Liu C., Parmigiani G., Park B.H., Bachman K.E., Papadopoulos N., Vogelstein B., Kinzler K.W., Velculescu V.E. Science 314:268-274(2006) [PubMed] [Europe PMC] [Abstract] Cited for: VARIANT [LARGE SCALE ANALYSIS] TRP-562.
+	Additional computationally mapped references.

Cross-references

Sequence databases
EMBL GenBank DDBJ	DQ020495 mRNA. Translation: AAY84832.1. AK127206 mRNA. Translation: BAC86883.1. AC104782 Genomic DNA. No translation available. AC012493 Genomic DNA. No translation available. BC101664 mRNA. Translation: AAI01665.1. BC101662 mRNA. Translation: AAI01663.1.
IPI	IPI00394812. IPI00827614.
RefSeq	NP_940863.3. NM_198461.3.
UniGene	Hs.21380.
3D structure databases
ProteinModelPortal	Q1L5Z9.
SMR	Q1L5Z9. Positions 447-509.
ModBase	Search...
Protein-protein interaction databases
IntAct	Q1L5Z9. 1 interaction.
STRING	9606.ENSP00000377086.
PTM databases
PhosphoSite	Q1L5Z9.
Polymorphism databases
DMDM	126215737.
Proteomic databases
PaxDb	Q1L5Z9.
PRIDE	Q1L5Z9.
Protocols and materials databases
StructuralBiologyKnowledgebase	Search...
Genome annotation databases
Ensembl	ENST00000393437; ENSP00000377086; ENSG00000170500. ENST00000409647; ENSP00000386823; ENSG00000170500.
GeneID	164832.
KEGG	hsa:164832.
UCSC	uc002tal.4. human.
Organism-specific databases
CTD	164832.
GeneCards	GC02M100889.
H-InvDB	HIX0024130.
HGNC	HGNC:24788. LONRF2.
neXtProt	NX_Q1L5Z9.
PharmGKB	PA142671540.
GenAtlas	Search...
Phylogenomic databases
eggNOG	COG2802.
HOGENOM	HOG000060199.
HOVERGEN	HBG081924.
InParanoid	Q1L5Z9.
OMA	KVMCEVL.
OrthoDB	EOG4BK538.
Gene expression databases
Bgee	Q1L5Z9.
CleanEx	HS_LONRF2.
Genevestigator	Q1L5Z9.
Family and domain databases
Gene3D	1.25.40.10. 2 hits. 3.30.40.10. 2 hits.
InterPro	IPR003111. Pept_S16_N. IPR015947. PUA-like_domain. IPR013026. TPR-contain_dom. IPR011990. TPR-like_helical. IPR019734. TPR_repeat. IPR001841. Znf_RING. IPR013083. Znf_RING/FYVE/PHD. IPR017907. Znf_RING_CS. [Graphical view]
Pfam	PF02190. LON. 1 hit. [Graphical view]
SMART	SM00184. RING. 2 hits. SM00028. TPR. 3 hits. [Graphical view]
SUPFAM	SSF88697. PUA-like. 1 hit.
PROSITE	PS50005. TPR. False negative. PS50293. TPR_REGION. 1 hit. PS00518. ZF_RING_1. 1 hit. PS50089. ZF_RING_2. 1 hit. [Graphical view]
ProtoNet	Search...
Other
ChiTaRS	LONRF2. human.
GenomeRNAi	164832.
NextBio	88502.

Entry information

Entry name

LONF2_HUMAN

Accession

Primary (citable) accession number: Q1L5Z9
Secondary accession number(s): B9A006, Q6ZSR4

Entry history

Integrated into UniProtKB/Swiss-Prot:	February 20, 2007
Last sequence update:	November 30, 2010
Last modified:	May 1, 2013
This is version 63 of the entry and version 3 of the sequence. [Complete history]

Entry status

Reviewed (UniProtKB/Swiss-Prot)

Annotation program

Chordata Protein Annotation Program

Disclaimer

Any medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.