Q17RB8 (LONF1_HUMAN) Reviewed, UniProtKB/Swiss-Prot

Names and origin

Protein names

Recommended name:
LON peptidase N-terminal domain and RING finger protein 1

Alternative name(s):
RING finger protein 191

Gene names

Name:	LONRF1
Synonyms:	RNF191

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	773 AA.
Sequence status	Complete.
Protein existence	Evidence at transcript level

General annotation (Comments)

Sequence similarities

Contains 1 Lon domain. Contains 2 RING-type zinc fingers. Contains 4 TPR repeats.

Sequence caution

The sequence AAI17382.1 differs from that shown. Reason: Erroneous initiation. The sequence AAI17386.1 differs from that shown. Reason: Erroneous initiation. The sequence BAB85052.1 differs from that shown. Reason: Frameshift at position 463. The sequence BAG59741.1 differs from that shown. Reason: Frameshift at position 293. The sequence BAG62246.1 differs from that shown. Reason: Erroneous initiation.

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: Q17RB8-1)

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: Q17RB8-2)

The sequence of this isoform differs from the canonical sequence as follows:
     452-462: Missing.

Sequence annotation (Features)

	Feature key	Position(s)	Length	Description	Graphical view	Feature identifier
Molecule processing
	Chain	1 – 773	773	LON peptidase N-terminal domain and RING finger protein 1		PRO_0000277669
Regions
	Repeat	48 – 81	34	TPR 1
	Repeat	212 – 244	33	TPR 2
	Repeat	246 – 278	33	TPR 3
	Repeat	279 – 312	34	TPR 4
	Domain	567 – 766	200	Lon
	Zinc finger	123 – 159	37	RING-type 1
	Zinc finger	479 – 517	39	RING-type 2
Natural variations
	Alternative sequence	452 – 462	11	Missing in isoform 2.		VSP_037971
	Natural variant	265	1	I → L. Corresponds to variant rs1139354 [ dbSNP | Ensembl ].		VAR_058706
Experimental info
	Sequence conflict	663	1	I → V in BAG62246. Ref.2
	Sequence conflict	773	1	K → E in BAG59741. Ref.2

Sequences

	Sequence		Length	Mass (Da)
	Isoform 1 [UniParc]. Last modified September 1, 2009. Version 2. Checksum: B151BEB07CF3638B	FASTA	773	86,725
	10 20 30 40 50 60 MSSPAVARTS PGGSREMAPA PQGRGRFWEV GGGSGHRLER AAAESERWEL LLRRGELLAL 70 80 90 100 110 120 GGHLKGALEA FAAALRRGAP ARPECLGALV DCLVFNYRLR HGLGWSAAPV AGADGGAGGL 130 140 150 160 170 180 LRCLGCRGFL SEPVTVPCGH SYCRRCLRRE LRARCRLCRD RLPPATASAT DAEGTAPRPP 190 200 210 220 230 240 PLAAAIAASD FRTSVVLNHL AEKWFPGQRE RARAAGRLGE LLHQGRYREA LAAACEALRA 250 260 270 280 290 300 EPSDLIVKIY RAESYAGLQE FKAAIEDLNA VLFQLPDWPE VYFRKGKVLC DAGFLGDALQ 310 320 330 340 350 360 LFLQCLALDE DFAPAKLQVQ KILCDLLLPE NLKEGLKESS WSSLPCTKNR PFDFHSVMEE 370 380 390 400 410 420 SQSLNEPSPK QSEEIPEVTS EPVKGSLNRA QSAQSINSTE MPAREDCLKR VSSEPVLSVQ 430 440 450 460 470 480 EKGVLLKRKL SLLEQDVIVN EDGRNKLKKQ GETPNEVCMF SLAYGDIPEE LIDVSDFECS 490 500 510 520 530 540 LCMRLFFEPV TTPCGHSFCK NCLERCLDHA PYCPLCKESL KEYLADRRYC VTQLLEELIV 550 560 570 580 590 600 KYLPDELSER KKIYDEETAE LSHLTKNVPI FVCTMAYPTV PCPLHVFEPR YRLMIRRSIQ 610 620 630 640 650 660 TGTKQFGMCV SDTQNSFADY GCMLQIRNVH FLPDGRSVVD TVGGKRFRVL KRGMKDGYCT 670 680 690 700 710 720 ADIEYLEDVK VENEDEIKNL RELHDLVYSQ ACSWFQNLRD RFRSQILQHF GSMPEREENL 730 740 750 760 770 QAAPNGPAWC WWLLAVLPVD PRYQLSVLSM KSLKERLTKI QHILTYFSRD QSK
	Isoform 2 [UniParc]. Checksum: A9A06A836F433EE5	FASTA	762	85,473
	10 20 30 40 50 60 MSSPAVARTS PGGSREMAPA PQGRGRFWEV GGGSGHRLER AAAESERWEL LLRRGELLAL 70 80 90 100 110 120 GGHLKGALEA FAAALRRGAP ARPECLGALV DCLVFNYRLR HGLGWSAAPV AGADGGAGGL 130 140 150 160 170 180 LRCLGCRGFL SEPVTVPCGH SYCRRCLRRE LRARCRLCRD RLPPATASAT DAEGTAPRPP 190 200 210 220 230 240 PLAAAIAASD FRTSVVLNHL AEKWFPGQRE RARAAGRLGE LLHQGRYREA LAAACEALRA 250 260 270 280 290 300 EPSDLIVKIY RAESYAGLQE FKAAIEDLNA VLFQLPDWPE VYFRKGKVLC DAGFLGDALQ 310 320 330 340 350 360 LFLQCLALDE DFAPAKLQVQ KILCDLLLPE NLKEGLKESS WSSLPCTKNR PFDFHSVMEE 370 380 390 400 410 420 SQSLNEPSPK QSEEIPEVTS EPVKGSLNRA QSAQSINSTE MPAREDCLKR VSSEPVLSVQ 430 440 450 460 470 480 EKGVLLKRKL SLLEQDVIVN EDGRNKLKKQ GAYGDIPEEL IDVSDFECSL CMRLFFEPVT 490 500 510 520 530 540 TPCGHSFCKN CLERCLDHAP YCPLCKESLK EYLADRRYCV TQLLEELIVK YLPDELSERK 550 560 570 580 590 600 KIYDEETAEL SHLTKNVPIF VCTMAYPTVP CPLHVFEPRY RLMIRRSIQT GTKQFGMCVS 610 620 630 640 650 660 DTQNSFADYG CMLQIRNVHF LPDGRSVVDT VGGKRFRVLK RGMKDGYCTA DIEYLEDVKV 670 680 690 700 710 720 ENEDEIKNLR ELHDLVYSQA CSWFQNLRDR FRSQILQHFG SMPEREENLQ AAPNGPAWCW 730 740 750 760 WLLAVLPVDP RYQLSVLSMK SLKERLTKIQ HILTYFSRDQ SK

References

[1]	"DNA sequence and analysis of human chromosome 8." Nusbaum C., Mikkelsen T.S., Zody M.C., Asakawa S., Taudien S., Garber M., Kodira C.D., Schueler M.G., Shimizu A., Whittaker C.A., Chang J.L., Cuomo C.A., Dewar K., FitzGerald M.G., Yang X., Allen N.R., Anderson S., Asakawa T. , Blechschmidt K., Bloom T., Borowsky M.L., Butler J., Cook A., Corum B., DeArellano K., DeCaprio D., Dooley K.T., Dorris L. III, Engels R., Gloeckner G., Hafez N., Hagopian D.S., Hall J.L., Ishikawa S.K., Jaffe D.B., Kamat A., Kudoh J., Lehmann R., Lokitsang T., Macdonald P., Major J.E., Matthews C.D., Mauceli E., Menzel U., Mihalev A.H., Minoshima S., Murayama Y., Naylor J.W., Nicol R., Nguyen C., O'Leary S.B., O'Neill K., Parker S.C.J., Polley A., Raymond C.K., Reichwald K., Rodriguez J., Sasaki T., Schilhabel M., Siddiqui R., Smith C.L., Sneddon T.P., Talamas J.A., Tenzin P., Topham K., Venkataraman V., Wen G., Yamazaki S., Young S.K., Zeng Q., Zimmer A.R., Rosenthal A., Birren B.W., Platzer M., Shimizu N., Lander E.S. Nature 439:331-335(2006) [PubMed] [Europe PMC] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
[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] OF 268-773 (ISOFORM 1), NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] OF 387-773 (ISOFORM 2). Tissue: Hepatoma.
[3]	"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] OF 337-773 (ISOFORM 1). Tissue: Brain and Ovary.

Cross-references

Sequence databases
EMBL GenBank DDBJ	AC123777 Genomic DNA. No translation available. AK074329 mRNA. Translation: BAB85052.1. Frameshift. AK297266 mRNA. Translation: BAG59741.1. Frameshift. AK300538 mRNA. Translation: BAG62246.1. Different initiation. BC004538 mRNA. Translation: AAH04538.2. BC117381 mRNA. Translation: AAI17382.1. Different initiation. BC117385 mRNA. Translation: AAI17386.1. Different initiation.
IPI	IPI00291662. IPI00872653.
RefSeq	NP_689484.3. NM_152271.3.
UniGene	Hs.180178.
3D structure databases
ProteinModelPortal	Q17RB8.
ModBase	Search...
Protein-protein interaction databases
IntAct	Q17RB8. 3 interactions.
STRING	9606.ENSP00000381298.
PTM databases
PhosphoSite	Q17RB8.
Polymorphism databases
DMDM	257051033.
Proteomic databases
PaxDb	Q17RB8.
PRIDE	Q17RB8.
Protocols and materials databases
StructuralBiologyKnowledgebase	Search...
Genome annotation databases
Ensembl	ENST00000398246; ENSP00000381298; ENSG00000154359. ENST00000533751; ENSP00000432130; ENSG00000154359.
GeneID	91694.
KEGG	hsa:91694.
UCSC	uc003wwd.1. human.
Organism-specific databases
CTD	91694.
GeneCards	GC08M012579.
HGNC	HGNC:26302. LONRF1.
HPA	HPA023584. HPA024814.
neXtProt	NX_Q17RB8.
PharmGKB	PA128394743.
GenAtlas	Search...
Phylogenomic databases
eggNOG	COG2802.
HOGENOM	HOG000060199.
HOVERGEN	HBG081924.
InParanoid	Q17RB8.
OMA	GHSYCRR.
OrthoDB	EOG4WQ12B.
Gene expression databases
ArrayExpress	Q17RB8.
Bgee	Q17RB8.
CleanEx	HS_LONRF1.
Genevestigator	Q17RB8.
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. IPR013105. TPR_2. IPR019734. TPR_repeat. IPR018957. Znf_C3HC4_RING-type. IPR001841. Znf_RING. IPR013083. Znf_RING/FYVE/PHD. IPR017907. Znf_RING_CS. [Graphical view]
Pfam	PF02190. LON. 1 hit. PF07719. TPR_2. 1 hit. PF00097. zf-C3HC4. 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. 2 hits. PS50089. ZF_RING_2. 2 hits. [Graphical view]
ProtoNet	Search...
Other
GenomeRNAi	91694.
NextBio	77403.

Entry information

Entry name

LONF1_HUMAN

Accession

Primary (citable) accession number: Q17RB8
Secondary accession number(s): B4DM29 , B4DU84, Q8TEA0, Q9BSV1

Entry history

Integrated into UniProtKB/Swiss-Prot:	February 20, 2007
Last sequence update:	September 1, 2009
Last modified:	May 1, 2013
This is version 66 of the entry and version 2 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.