Reviewed, UniProtKB/Swiss-Prot Q8CG71 (P3H2_MOUSE)

Names and origin

Protein names

Recommended name:
    Prolyl 3-hydroxylase 2
    EC=1.14.11.7
Alternative name(s):
    Leprecan-like protein 1

Gene names

Name:	Leprel1
Synonyms:	P3h2

Organism

Mus musculus (Mouse)

Taxonomic identifier

10090 [NCBI]

Taxonomic lineage

Eukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Euarchontoglires › Glires › Rodentia › Sciurognathi › Muroidea › Muridae › Murinae › Mus

Protein attributes

Sequence length	703 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	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 By similarity.
Catalytic activity	Procollagen L-proline + 2-oxoglutarate + O(2) = procollagen trans-3-hydroxy-L-proline + succinate + CO(2).
Cofactor	Iron By similarity. Ascorbate By similarity.
Subcellular location	Endoplasmic reticulum. Golgi apparatusBy similarity.
Sequence similarities	Belongs to the leprecan family. Contains 1 PKHD (prolyl/lysyl hydroxylase) domain. Contains 4 TPR repeats.

Ontologies

Keywords
Cellular component	Endoplasmic reticulum Golgi apparatus
Domain	Repeat Signal TPR repeat
Ligand	Iron Metal-binding Vitamin C
Molecular function	Dioxygenase Oxidoreductase
PTM	Glycoprotein
Gene Ontology (GO)
Biological process	oxidation reduction Inferred from electronic annotation. Source: UniProtKB-KW protein metabolic process Inferred from electronic annotation. Source: InterPro
Cellular component	Golgi apparatus Inferred from electronic annotation. Source: UniProtKB-KW endoplasmic reticulum Inferred from electronic annotation. Source: InterPro
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 electronic annotation. Source: EC
Complete GO annotation...

Sequence annotation (Features)

	Feature key	Position(s)	Length	Description	Graphical view	Feature identifier
Molecule processing
	Signal peptide	1 – 21	21	Potential
	Chain	22 – 703	682	Prolyl 3-hydroxylase 2		PRO_0000240357
Regions
	Repeat	42 – 75	34	TPR 1
	Repeat	144 – 177	34	TPR 2
	Repeat	205 – 238	34	TPR 3
	Repeat	301 – 334	34	TPR 4
	Domain	457 – 665	209	PKHD
	Motif	700 – 703	4	Prevents secretion from ER Potential
Sites
	Active site	657	1	By similarity
	Metal binding	575	1	Iron
	Metal binding	577	1	Iron
	Metal binding	647	1	Iron
Amino acid modifications
	Glycosylation	444	1	N-linked (GlcNAc...) Potential
	Glycosylation	544	1	N-linked (GlcNAc...) Potential
Experimental info
	Sequence conflict	497	1	P → A in BAC36342. Ref.2

Sequences

	Sequence		Length	Mass (Da)
	Q8CG71-1 [UniParc]. Last modified March 1, 2003. Version 1. Checksum: 1F42F9B9938573E4	FASTA	703	80,154
	10 20 30 40 50 60 MRESTWVSLL LLLLLPTPQR GGPQDGRRSP EPEPERGPLQ PFDLLYASGV AAYYSGDYER 70 80 90 100 110 120 AVRDLEAALS SHRRLRDIRT RCARHCAARR PLAPPGAGPG AELPFFRAVL ERARCSRSCQ 130 140 150 160 170 180 SQRLGGPASR HRVSEDVRSD FQRRVPYNYL QRAYIKLNQL EKAMEAAHTF FMANPEHMEM 190 200 210 220 230 240 QQDLEDYKAT ARVEAPLLDR EAKPHLESYN AGVKHYEADD FESAIKYFEQ ALREYFNEDM 250 260 270 280 290 300 ECRALCEGPQ RFEEYEYLGY KGGLYEAIAD HYMQVLVCQH ECVRELATRP GRLSPIENFL 310 320 330 340 350 360 PLHYDYLQFA YYRVGEYVKA LECAKAYLMF HPDNEDVLDN VDFYESLLDD STDPASIEAR 370 380 390 400 410 420 EDLTAFVKRH KLEAELIKLA AEGLGFSYAE PNYWISYGGR QDENRVPSGV NMDGAEVHGL 430 440 450 460 470 480 SMGKKSPPKI GRDLREGGPL LYENITFVYN SEQLNGTQRV LLDNVLSQEQ CRELHSVANG 490 500 510 520 530 540 IMLVGDGYRG KTSPHTPNEK FEGATVLKAL KFGYEGRVPL KSARLFYDIS EKARKIVESY 550 560 570 580 590 600 FMLNSTLYFS YTHMVCRTAL SGQQDRRNDL SHPIHADNCL LDPEANECWK EPPAYTFRDY 610 620 630 640 650 660 SALLYMNDDF DGGEFIFTEM DAKTVTASIK PKCGRMISFS SGGENPHGVK AVTRGQRCAV 670 680 690 700 ALWFTLDPLY RELERIQADE VIAILDQEQR GKHGLNINPK DEL

References

	« Hide 'large scale' referencesShow 'large scale' references »
[1]	"LEPREL1, a novel ER and Golgi resident member of the Leprecan family." Jaernum S., Kjellman C., Darabi A., Nilsson I., Edvardsen K., Aaman P. Biochem. Biophys. Res. Commun. 317:342-351(2004) [PubMed: 15063763] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [MRNA]. Strain: C57BL/6.
[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: 16141072] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] OF 295-703. Strain: C57BL/6J. Tissue: Head.

Cross-references

Sequence databases
EMBLGenBankDDBJ	AJ430350 mRNA. Translation: CAD23038.1. AK076434 mRNA. Translation: BAC36342.1.
RefSeq	NP_775555.1.
UniGene	Mm.326869
3D structure databases
ModBase	Search...
PTM databases
PhosphoSite	Q8CG71.
Genome annotation databases
Ensembl	ENSMUSG00000038168. Mus musculus. [Contig view]
GeneID	210530.
KEGG	mmu:210530.
Organism-specific databases
MGI	MGI:2146663. Leprel1.
Phylogenomic databases
HOVERGEN	Q8CG71.
Gene expression databases
ArrayExpress	Q8CG71.
CleanEx	MM_LEPREL1.
GermOnline	ENSMUSG00000038168. Mus musculus.
Family and domain databases
InterPro	IPR005123. 2OG-FeII_Oase. IPR000886. ER_targeting_sequence. IPR006620. Pro_4_hyd_alph. IPR013105. TPR_2. IPR013026. TPR_region. [Graphical view]
Pfam	PF03171. 2OG-FeII_Oxy. 1 hit. PF07719. TPR_2. 2 hits. [Graphical view]
SMART	SM00702. P4Hc. 1 hit. [Graphical view]
PROSITE	PS00014. ER_TARGET. 1 hit. PS50005. TPR. False negative. PS50293. TPR_REGION. False negative. [Graphical view]
ProtoNet	Search...
Other Resources
NextBio	372972.
SOURCE	Search...

Entry information

Entry name

P3H2_MOUSE

Accession

Primary (citable) accession number: Q8CG71
Secondary accession number(s): Q8C673

Entry history

Integrated into UniProtKB/Swiss-Prot:	June 27, 2006
Last sequence update:	March 1, 2003
Last modified:	November 25, 2008
This is version 42 of the entry and version 1 of the sequence. [Complete history]

Entry status

Reviewed (UniProtKB/Swiss-Prot)

Annotation project

HPI (Human Proteome Initiative)