Q3V1T4 (P3H1_MOUSE) Reviewed, UniProtKB/Swiss-Prot

Names and origin

Protein names

Recommended name:
Prolyl 3-hydroxylase 1
EC=1.14.11.7

Alternative name(s):
Growth suppressor 1
Leucine- and proline-enriched proteoglycan 1
Short name=Leprecan-1

Gene names

Name:	Lepre1
Synonyms:	Gros1, P3h1

Organism

Mus musculus (Mouse) [Reference proteome]

Taxonomic identifier

10090 [NCBI]

Taxonomic lineage

cellular organisms › Eukaryota › Opisthokonta › Metazoa › Eumetazoa › Bilateria › Deuterostomia › Chordata › Craniata › Vertebrata › Gnathostomata › Teleostomi › Euteleostomi › Sarcopterygii › Tetrapoda › Amniota › Mammalia › Theria › Eutheria › Euarchontoglires › Glires › Rodentia › Sciurognathi › Muroidea › Muridae › Murinae › Mus › Mus

Protein attributes

Sequence length	739 AA.
Sequence status	Complete.
Sequence processing	The displayed sequence is further processed into a mature form.
Protein existence	Evidence at transcript level

General annotation (Comments)

Function	Basement membrane-associated chondroitin sulfate proteoglycan (CSPG). Has prolyl 3-hydroxylase activity catalyzing the post-translational formation of 3-hydroxyproline in -Xaa-Pro-Gly- sequences in collagens, especially types IV and V. May be involved in the secretory pathway of cells. Has growth suppressive activity in fibroblasts By similarity.
Catalytic activity	L-proline-[procollagen] + 2-oxoglutarate + O2 = trans-3-hydroxy-L-proline-[procollagen] + succinate + CO2.
Cofactor	Iron By similarity. Ascorbate By similarity.
Subcellular location	Endoplasmic reticulum By similarity. Secreted › extracellular space › extracellular matrix By similarity. Note: Secreted into the extracellular matrix as a chondroitin sulfate proteoglycan (CSPG).
Post-translational modification	O-glycosylated; chondroitin sulfate By similarity.
Sequence similarities	Belongs to the leprecan family. Contains 1 Fe2OG dioxygenase domain. Contains 4 TPR repeats.
Sequence caution	The sequence BAB27041.1 differs from that shown. Reason: Frameshift at position 707. The sequence BAE21065.1 differs from that shown. Reason: Intron retention.

Ontologies

Keywords
Cellular component	Endoplasmic reticulum Extracellular matrix Secreted
Coding sequence diversity	Alternative splicing
Domain	Coiled coil Repeat Signal TPR repeat
Ligand	Iron Metal-binding Vitamin C
Molecular function	Dioxygenase Oxidoreductase
PTM	Glycoprotein
Technical term	Complete proteome Reference proteome
Gene Ontology (GO)
Biological_process	cell growth Inferred from direct assay Ref.1. Source: MGI collagen fibril organization Inferred from mutant phenotype PubMed 20363744. Source: MGI collagen metabolic process Inferred from sequence orthology PubMed 15044469. Source: MGI regulation of ossification Inferred from mutant phenotype PubMed 20363744. Source: MGI
Cellular_component	endoplasmic reticulum Inferred from sequence orthology PubMed 15044469. Source: MGI nucleus Inferred from direct assay Ref.1. Source: MGI plasma membrane Traceable author statement Ref.1. Source: MGI proteinaceous extracellular matrix Inferred from electronic annotation. Source: UniProtKB-SubCell
Molecular_function	L-ascorbic acid binding Inferred from electronic annotation. Source: UniProtKB-KW iron ion binding Inferred from electronic annotation. Source: InterPro oxidoreductase activity, acting on single donors with incorporation of molecular oxygen, incorporation of two atoms of oxygen Inferred from electronic annotation. Source: UniProtKB-KW procollagen-proline 3-dioxygenase activity Inferred from sequence orthology PubMed 15044469. Source: MGI
Complete GO annotation...

Alternative products

This entry describes 3 isoforms produced by alternative splicing. [Align] [Select]

Isoform 1 (identifier: Q3V1T4-1)

Also known as: GROS1-L;

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: Q3V1T4-2)

Also known as: GROS1-S;

The sequence of this isoform differs from the canonical sequence as follows:
     540-543: MYYN → TALQ
     544-739: Missing.

Isoform 3 (identifier: Q3V1T4-3)

The sequence of this isoform differs from the canonical sequence as follows:
     1-179: Missing.

Note: No experimental confirmation available.

Sequence annotation (Features)

	Feature key	Position(s)	Length	Description	Graphical view	Feature identifier
Molecule processing
	Signal peptide	1 – 25	25	Potential
	Chain	26 – 739	714	Prolyl 3-hydroxylase 1		PRO_0000240353
Regions
	Repeat	36 – 69	34	TPR 1
	Repeat	146 – 179	34	TPR 2
	Repeat	208 – 241	34	TPR 3
	Repeat	304 – 337	34	TPR 4
	Domain	567 – 681	115	Fe2OG dioxygenase
	Coiled coil	404 – 442	39	Potential
	Motif	736 – 739	4	Prevents secretion from ER Potential
Sites
	Active site	672	1	By similarity
	Metal binding	590	1	Iron
	Metal binding	592	1	Iron
	Metal binding	662	1	Iron
Amino acid modifications
	Glycosylation	319	1	N-linked (GlcNAc...) Potential
	Glycosylation	470	1	N-linked (GlcNAc...) Potential
	Glycosylation	543	1	N-linked (GlcNAc...) Potential
Natural variations
	Alternative sequence	1 – 179	179	Missing in isoform 3.		VSP_019349
	Alternative sequence	540 – 543	4	MYYN → TALQ in isoform 2.		VSP_019350
	Alternative sequence	544 – 739	196	Missing in isoform 2.		VSP_019351
Experimental info
	Sequence conflict	4 – 25	22	SERRL…LRVAA → TKGGCWHDASGRRRRRLTGC G in AAF04806. Ref.1
	Sequence conflict	4 – 25	22	SERRL…LRVAA → TKGGCWHDASGRRRRRLTGC G in AAF04807. Ref.1
	Sequence conflict	50	1	G → R in AAF04806. Ref.1
	Sequence conflict	50	1	G → R in AAF04807. Ref.1
	Sequence conflict	371 – 372	2	RS → PN in AAF04806. Ref.1
	Sequence conflict	371 – 372	2	RS → PN in AAF04807. Ref.1
	Sequence conflict	403	1	P → T in BAE21065. Ref.2
	Sequence conflict	420	1	Missing in BAC26962. Ref.2
	Sequence conflict	484	1	D → N in BAE35138. Ref.2
	Sequence conflict	569	1	L → F in AAF04806. Ref.1
	Sequence conflict	589	1	V → D in BAE21065. Ref.2
	Sequence conflict	601	1	L → F in AAF04806. Ref.1
	Sequence conflict	614	1	D → E in AAF04806. Ref.1
	Sequence conflict	685	1	H → Q in BAC26962. Ref.2
	Sequence conflict	716	1	D → G in BAE35138. Ref.2
	Sequence conflict	716	1	D → G in AAH24047. Ref.3
	Sequence conflict	727 – 739	13	SLSDR…HKDEL → FLHGATVLGVGIA in AAF04806. Ref.1

Sequences

	Sequence		Length	Mass (Da)
	Isoform 1 (GROS1-L) [UniParc]. Last modified June 27, 2006. Version 2. Checksum: 3484AE68E80B68E8	FASTA	739	83,651
	10 20 30 40 50 60 MAVSERRLLA AMLAVAAAAA LRVAAESEPG WDVAAPDLLY AEGTAAYSRG DWPGVVLNME 70 80 90 100 110 120 RALRSRAALR ALRLRCRTRC ATELPWAPDL DLGPDPSLSQ DPGAAALHDL RFFGAVLRRA 130 140 150 160 170 180 ACLRRCLGPP SAHLLSEELD LEFNKRSPYN YLQVAYFKIN KLEKAVAAAH TFFVGNPEHM 190 200 210 220 230 240 EMRQNLDYYQ TMSGVKEADF RDLEAKPHMH EFRLGVRLYS EEKPQEAVPH LEAALQEYFV 250 260 270 280 290 300 ADEECRALCE GPYDYDGYNY LDYSADLFQA ITDHYVQVLN CKQNCVTELA SHPSREKPFE 310 320 330 340 350 360 DFLPSHYNYL QFAYYNIGNY TQAIECAKTY LLFFPNDEVM HQNLAYYTAM LGEEEASSIS 370 380 390 400 410 420 PRENAEEYRR RSLLEKELLF FAYDIFGIPF VDPDSWTPEE VIPKRLQEKQ KSERETAVRI 430 440 450 460 470 480 SQEIGNLMKE IETLVEEKTK ESLDVSRLTR EGGPLLYEGI SLTMNSKVLN GSQRVVMDGV 490 500 510 520 530 540 ISDDECQELQ RLTNAAATSG DGYRGQTSPH TPNEKFYGVT VLKALKLGQE GKVPLQSARM 550 560 570 580 590 600 YYNVTEKVRR VMESYFRLDT PLYFSYSHLV CRTAIEESQA ERKDSSHPVH VDNCILNAEA 610 620 630 640 650 660 LMCIKEPPAY TFRDYSAILY LNGDFDGGNF YFTELDAKTV TAEVQPQCGR AVGFSSGTEN 670 680 690 700 710 720 PHGVKAVTRG QRCAIALWFT LDPRHSERDR VQADDLVKML FSPEEVDLPQ EQPLPDQQGS 730 PEPGEESLSD RGSLHKDEL
	Isoform 2 (GROS1-S) [UniParc]. Checksum: 2166EB32EC38A766	FASTA	543	61,397
	10 20 30 40 50 60 MAVSERRLLA AMLAVAAAAA LRVAAESEPG WDVAAPDLLY AEGTAAYSRG DWPGVVLNME 70 80 90 100 110 120 RALRSRAALR ALRLRCRTRC ATELPWAPDL DLGPDPSLSQ DPGAAALHDL RFFGAVLRRA 130 140 150 160 170 180 ACLRRCLGPP SAHLLSEELD LEFNKRSPYN YLQVAYFKIN KLEKAVAAAH TFFVGNPEHM 190 200 210 220 230 240 EMRQNLDYYQ TMSGVKEADF RDLEAKPHMH EFRLGVRLYS EEKPQEAVPH LEAALQEYFV 250 260 270 280 290 300 ADEECRALCE GPYDYDGYNY LDYSADLFQA ITDHYVQVLN CKQNCVTELA SHPSREKPFE 310 320 330 340 350 360 DFLPSHYNYL QFAYYNIGNY TQAIECAKTY LLFFPNDEVM HQNLAYYTAM LGEEEASSIS 370 380 390 400 410 420 PRENAEEYRR RSLLEKELLF FAYDIFGIPF VDPDSWTPEE VIPKRLQEKQ KSERETAVRI 430 440 450 460 470 480 SQEIGNLMKE IETLVEEKTK ESLDVSRLTR EGGPLLYEGI SLTMNSKVLN GSQRVVMDGV 490 500 510 520 530 540 ISDDECQELQ RLTNAAATSG DGYRGQTSPH TPNEKFYGVT VLKALKLGQE GKVPLQSART ALQ
	Isoform 3 [UniParc]. Checksum: 673B7643F80A1388	FASTA	560	64,102
	10 20 30 40 50 60 MEMRQNLDYY QTMSGVKEAD FRDLEAKPHM HEFRLGVRLY SEEKPQEAVP HLEAALQEYF 70 80 90 100 110 120 VADEECRALC EGPYDYDGYN YLDYSADLFQ AITDHYVQVL NCKQNCVTEL ASHPSREKPF 130 140 150 160 170 180 EDFLPSHYNY LQFAYYNIGN YTQAIECAKT YLLFFPNDEV MHQNLAYYTA MLGEEEASSI 190 200 210 220 230 240 SPRENAEEYR RRSLLEKELL FFAYDIFGIP FVDPDSWTPE EVIPKRLQEK QKSERETAVR 250 260 270 280 290 300 ISQEIGNLMK EIETLVEEKT KESLDVSRLT REGGPLLYEG ISLTMNSKVL NGSQRVVMDG 310 320 330 340 350 360 VISDDECQEL QRLTNAAATS GDGYRGQTSP HTPNEKFYGV TVLKALKLGQ EGKVPLQSAR 370 380 390 400 410 420 MYYNVTEKVR RVMESYFRLD TPLYFSYSHL VCRTAIEESQ AERKDSSHPV HVDNCILNAE 430 440 450 460 470 480 ALMCIKEPPA YTFRDYSAIL YLNGDFDGGN FYFTELDAKT VTAEVQPQCG RAVGFSSGTE 490 500 510 520 530 540 NPHGVKAVTR GQRCAIALWF TLDPRHSERD RVQADDLVKM LFSPEEVDLP QEQPLPDQQG 550 560 SPEPGEESLS DRGSLHKDEL

References

	« Hide 'large scale' referencesShow 'large scale' references »
[1]	"Gros1, a potential growth suppressor on chromosome 1: its identity to basement membrane-associated proteoglycan, leprecan." Kaul S.C., Sugihara T., Yoshida A., Nomura H., Wadhwa R. Oncogene 19:3576-3583(2000) [PubMed] [Europe PMC] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [MRNA] (ISOFORMS 1 AND 2). Strain: CD-1/ICR. Tissue: Fibroblast and Testis.
[2]	"The transcriptional landscape of the mammalian genome." Carninci P., Kasukawa T., Katayama S., Gough J., Frith M.C., Maeda N., Oyama R., Ravasi T., Lenhard B., Wells C., Kodzius R., Shimokawa K., Bajic V.B., Brenner S.E., Batalov S., Forrest A.R., Zavolan M., Davis M.J. , Wilming L.G., Aidinis V., Allen J.E., Ambesi-Impiombato A., Apweiler R., Aturaliya R.N., Bailey T.L., Bansal M., Baxter L., Beisel K.W., Bersano T., Bono H., Chalk A.M., Chiu K.P., Choudhary V., Christoffels A., Clutterbuck D.R., Crowe M.L., Dalla E., Dalrymple B.P., de Bono B., Della Gatta G., di Bernardo D., Down T., Engstrom P., Fagiolini M., Faulkner G., Fletcher C.F., Fukushima T., Furuno M., Futaki S., Gariboldi M., Georgii-Hemming P., Gingeras T.R., Gojobori T., Green R.E., Gustincich S., Harbers M., Hayashi Y., Hensch T.K., Hirokawa N., Hill D., Huminiecki L., Iacono M., Ikeo K., Iwama A., Ishikawa T., Jakt M., Kanapin A., Katoh M., Kawasawa Y., Kelso J., Kitamura H., Kitano H., Kollias G., Krishnan S.P., Kruger A., Kummerfeld S.K., Kurochkin I.V., Lareau L.F., Lazarevic D., Lipovich L., Liu J., Liuni S., McWilliam S., Madan Babu M., Madera M., Marchionni L., Matsuda H., Matsuzawa S., Miki H., Mignone F., Miyake S., Morris K., Mottagui-Tabar S., Mulder N., Nakano N., Nakauchi H., Ng P., Nilsson R., Nishiguchi S., Nishikawa S., Nori F., Ohara O., Okazaki Y., Orlando V., Pang K.C., Pavan W.J., Pavesi G., Pesole G., Petrovsky N., Piazza S., Reed J., Reid J.F., Ring B.Z., Ringwald M., Rost B., Ruan Y., Salzberg S.L., Sandelin A., Schneider C., Schoenbach C., Sekiguchi K., Semple C.A., Seno S., Sessa L., Sheng Y., Shibata Y., Shimada H., Shimada K., Silva D., Sinclair B., Sperling S., Stupka E., Sugiura K., Sultana R., Takenaka Y., Taki K., Tammoja K., Tan S.L., Tang S., Taylor M.S., Tegner J., Teichmann S.A., Ueda H.R., van Nimwegen E., Verardo R., Wei C.L., Yagi K., Yamanishi H., Zabarovsky E., Zhu S., Zimmer A., Hide W., Bult C., Grimmond S.M., Teasdale R.D., Liu E.T., Brusic V., Quackenbush J., Wahlestedt C., Mattick J.S., Hume D.A., Kai C., Sasaki D., Tomaru Y., Fukuda S., Kanamori-Katayama M., Suzuki M., Aoki J., Arakawa T., Iida J., Imamura K., Itoh M., Kato T., Kawaji H., Kawagashira N., Kawashima T., Kojima M., Kondo S., Konno H., Nakano K., Ninomiya N., Nishio T., Okada M., Plessy C., Shibata K., Shiraki T., Suzuki S., Tagami M., Waki K., Watahiki A., Okamura-Oho Y., Suzuki H., Kawai J., Hayashizaki Y. Science 309:1559-1563(2005) [PubMed] [Europe PMC] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORMS 1 AND 3). Strain: C57BL/6J. Tissue: Embryonic stem cell, Pituitary and Placenta.
[3]	"Lineage-specific biology revealed by a finished genome assembly of the mouse." Church D.M., Goodstadt L., Hillier L.W., Zody M.C., Goldstein S., She X., Bult C.J., Agarwala R., Cherry J.L., DiCuccio M., Hlavina W., Kapustin Y., Meric P., Maglott D., Birtle Z., Marques A.C., Graves T., Zhou S. , Teague B., Potamousis K., Churas C., Place M., Herschleb J., Runnheim R., Forrest D., Amos-Landgraf J., Schwartz D.C., Cheng Z., Lindblad-Toh K., Eichler E.E., Ponting C.P. PLoS Biol. 7:E1000112-E1000112(2009) [PubMed] [Europe PMC] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA]. Strain: C57BL/6J.
[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 1). Strain: FVB/N. Tissue: Mammary tumor.
+	Additional computationally mapped references.

Cross-references

Sequence databases
EMBL GenBank DDBJ	AF165163 mRNA. Translation: AAF04806.1. Sequence problems. AF165164 mRNA. Translation: AAF04807.1. Sequence problems. AK010578 mRNA. Translation: BAB27041.1. Frameshift. AK030436 mRNA. Translation: BAC26962.1. AK132262 mRNA. Translation: BAE21065.1. Sequence problems. AK159505 mRNA. Translation: BAE35138.1. AL606975 Genomic DNA. Translation: CAM21645.1. AL606975 Genomic DNA. Translation: CAO78098.1. BC024047 mRNA. Translation: AAH24047.1.
IPI	IPI00109205. IPI00762142. IPI00762805.
RefSeq	NP_001035874.1. NM_001042411.1. NP_062756.2. NM_019782.2. NP_062757.2. NM_019783.2.
UniGene	Mm.27961.
3D structure databases
ProteinModelPortal	Q3V1T4.
SMR	Q3V1T4. Positions 308-346.
ModBase	Search...
PTM databases
PhosphoSite	Q3V1T4.
Proteomic databases
PaxDb	Q3V1T4.
PRIDE	Q3V1T4.
Protocols and materials databases
StructuralBiologyKnowledgebase	Search...
Genome annotation databases
Ensembl	ENSMUST00000081606; ENSMUSP00000080312; ENSMUSG00000028641. ENSMUST00000121111; ENSMUSP00000112504; ENSMUSG00000028641.
GeneID	56401.
KEGG	mmu:56401.
UCSC	uc008ulq.1. mouse. uc008ulr.1. mouse.
Organism-specific databases
CTD	64175.
MGI	MGI:1888921. Lepre1.
Phylogenomic databases
eggNOG	NOG269251.
GeneTree	ENSGT00550000074573.
HOVERGEN	HBG053224.
KO	K08134.
OrthoDB	EOG43XV34.
Gene expression databases
ArrayExpress	Q3V1T4.
Bgee	Q3V1T4.
CleanEx	MM_LEPRE1.
Genevestigator	Q3V1T4.
GermOnline	ENSMUSG00000028641. Mus musculus.
Family and domain databases
Gene3D	1.25.40.10. 3 hits.
InterPro	IPR005123. Oxoglu/Fe-dep_dioxygenase. IPR006620. Pro_4_hyd_alph. IPR011990. TPR-like_helical. [Graphical view]
Pfam	PF13640. 2OG-FeII_Oxy_3. 1 hit. [Graphical view]
SMART	SM00702. P4Hc. 1 hit. [Graphical view]
PROSITE	PS00014. ER_TARGET. 1 hit. PS51471. FE2OG_OXY. 1 hit. PS50005. TPR. False negative. PS50293. TPR_REGION. False negative. [Graphical view]
ProtoNet	Search...
Other
NextBio	312512.
SOURCE	Search...

Entry information

Entry name

P3H1_MOUSE

Accession

Primary (citable) accession number: Q3V1T4
Secondary accession number(s): A2A7Q4 , A6PW85, Q3TWX8, Q8BSV2, Q8CFL3, Q9CWK5, Q9QZT6, Q9QZT7

Entry history

Integrated into UniProtKB/Swiss-Prot:	June 27, 2006
Last sequence update:	June 27, 2006
Last modified:	May 1, 2013
This is version 73 of the entry and version 2 of the sequence. [Complete history]

Entry status

Reviewed (UniProtKB/Swiss-Prot)

Annotation program

Chordata Protein Annotation Program