Reactome | SERPINH1 [endoplasmic reticulum lumen]

SERPINH1 [endoplasmic reticulum lumen]

Stable Identifier

R-HSA-2127480

Type

Protein [EntityWithAccessionedSequence]

Species

Homo sapiens

Compartment

endoplasmic reticulum lumen

Synonyms

Serpin H1, SERPH_HUMAN

Locations in the PathwayBrowser for interactor UniProt:P54252 ATXN3

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External Reference Information

External Reference

UniProt:P50454 SERPINH1

Gene Names

SERPINH1, CBP1, CBP2, HSP47, SERPINH2, PIG14

Chain

signal peptide:1-18, chain:19-418

Reference Genes

BioGPS Gene:871 SERPINH1

COSMIC (genes):SERPINH1 SERPINH1

CTD Gene:871 SERPINH1

dbSNP Gene:871 SERPINH1

ENSEMBL:ENSG00000149257 SERPINH1

ENSEMBL_homo_sapiens_GENE:ENSG00000149257.16 SERPINH1

HGNC:1546 SERPINH1

KEGG Gene (Homo sapiens):871 SERPINH1

Monarch:871 SERPINH1

NCBI Gene:871 SERPINH1

OMIM:600943 SERPINH1

UCSC:P50454 SERPINH1

Reference Transcript

Other Identifiers

11744209_s_at

11746320_x_at

11746321_s_at

16729128

207714_3p_s_at

207714_PM_s_at

207714_s_at

3167377

3340591

3340592

3340593

3340594

3340595

3340597

3340598

3340599

3340600

3340601

3340604

3340605

3340607

3340608

3340609

3340610

3340611

3340612

3340613

3340614

39166_s_at

39167_r_at

7942596

871

A_14_P201809

A_23_P76006

A_33_P3269203

D83174_s_at

GE57328

GE59869

GO:0003433

GO:0003723

GO:0004867

GO:0005515

GO:0005518

GO:0005576

GO:0005615

GO:0005737

GO:0005783

GO:0005788

GO:0005793

GO:0006986

GO:0030154

GO:0030198

GO:0030199

GO:0030312

GO:0031012

GO:0032964

GO:0043226

GO:0045121

GO:0048856

GO:0051082

GO:0051604

GO:0062023

GO:0098772

HMNXSV003012287

ILMN_1751028

PH_hs_0012962

PH_hs_0032271

TC11000809.hg

TC11002788.hg

Participates

as an output of

Secretion of collagens (Homo sapiens)

as a component of

as an input of

Procollagen triple helix formation (Homo sapiens)

Inferred To

Spn77Bb [endoplasmic reticulum lumen] (Drosophila melanogaster)

Spn28Dc [endoplasmic reticulum lumen] (Drosophila melanogaster)

Ghost homologue of SERPINH1 [endoplasmic reticulum lumen] (Caenorhabditis elegans)

Ghost homologue of SERPINH1 [endoplasmic reticulum lumen] (Dictyostelium discoideum)

Ghost homologue of SERPINH1 [endoplasmic reticulum lumen] (Xenopus tropicalis)

SERPINH1 [endoplasmic reticulum lumen] (Bos taurus)

SERPINH1 [endoplasmic reticulum lumen] (Canis familiaris)

SERPINH1 [endoplasmic reticulum lumen] (Gallus gallus)

SERPINH1 [endoplasmic reticulum lumen] (Sus scrofa)

Spn77Bc [endoplasmic reticulum lumen] (Drosophila melanogaster)

serpinh1b [endoplasmic reticulum lumen] (Danio rerio)

Serpinh1 [endoplasmic reticulum lumen] (Mus musculus)

Serpinh1 [endoplasmic reticulum lumen] (Rattus norvegicus)

Spn77Ba [endoplasmic reticulum lumen] (Drosophila melanogaster)

Homologues of SERPINH1 [endoplasmic reticulum lumen] (Drosophila melanogaster)

Cross References

RefSeq

NP_001193943.1, NP_001226.2, XP_011543629.1

OpenTargets

ENSG00000149257

ZINC - Substances

SERPH_HUMAN

HPA

ENSG00000149257-SERPINH1

GeneCards

P50454

ZINC target

P50454

Ensembl

ENSP00000350894, ENSP00000434657, ENST00000358171, ENST00000524558, ENSP00000434412, ENST00000533603, ENSG00000149257

PRO

P50454

Pharos - Targets

P50454

Orphanet

19046

ZINC - Predictions - Purchasable

SERPH_HUMAN

Interactors (40)

Accession	#Entities	Entities	Confidence Score	Evidence (IntAct)
UniProt:P13569 CFTR	17	PolyUb-CFTR [clathrin-coated endocytic vesicle membrane] (R-HSA-8869104) PolyUb-CFTR [plasma membrane] (R-HSA-8867595) CFTR [lysosomal membrane] (R-HSA-5627279) CFTR [Golgi-associated vesicle membrane] (R-HSA-5627079) CFTR [endosome membrane] (R-HSA-6782965) PolyUb-CFTR [endosome membrane] (R-HSA-6782976) CFTR F508del [endoplasmic reticulum membrane] (R-HSA-8866834) ub-CFTR F508del [cytosol] (R-HSA-8866838) ub-CFTR F508del [endoplasmic reticulum membrane] (R-HSA-8866842) CFTR G542* [plasma membrane] (R-HSA-5678986) CFTR G551D [plasma membrane] (R-HSA-5678977) CFTR W1282* [plasma membrane] (R-HSA-5678976) CFTR F508del [plasma membrane] (R-HSA-5678978) CFTR N1303K [plasma membrane] (R-HSA-5678983) CFTR [plasma membrane] (R-HSA-383188) CFTR [endoplasmic reticulum membrane] (R-HSA-8866837) misfolded CFTR [endoplasmic reticulum membrane] (R-HSA-8868730)	0.706	23
UniProt:Q13200 PSMD2	5	PSMD2 [secretory granule lumen] (R-HSA-6800919) PSMD2 [extracellular region] (R-HSA-6806479) PSMD2 [ficolin-1-rich granule lumen] (R-HSA-6800435) PSMD2 [nucleoplasm] (R-HSA-174311) PSMD2 [cytosol] (R-HSA-68796)	0.61	4
ChEBI:28304 HEPARIN			0.564	3
UniProt:Q8NE08 Q8NE08			0.556	3
UniProt:P61513 RPL37A	1	RPL37A [cytosol] (R-HSA-72468)	0.556	3
UniProt:Q16560-2 SNRNP35			0.556	3
UniProt:O60259 KLK8	3	KLK8 propeptide [extracellular region] (R-HSA-8850829) proKLK8 [extracellular region] (R-HSA-8850832) KLK8 [extracellular region] (R-HSA-8850833)	0.556	3
UniProt:Q969W3 VCF1			0.556	3
UniProt:Q8NCS7 SLC44A5	1	SLC44A5 [plasma membrane] (R-HSA-444459)	0.556	3
UniProt:O75874 IDH1	10	IDH1 R132H mutant [cytosol] (R-HSA-880015) IDH1 R132L mutant [cytosol] (R-HSA-880000) IDH1 R132S mutant [cytosol] (R-HSA-880048) IDH1 R132C mutant [cytosol] (R-HSA-880031) IDH1 [secretory granule lumen] (R-HSA-6800980) IDH1 [ficolin-1-rich granule lumen] (R-HSA-6800977) IDH1 [extracellular region] (R-HSA-6806433) IDH1 [tertiary granule lumen] (R-HSA-6800163) IDH1 [cytosol] (R-HSA-389549) IDH1 [peroxisomal matrix] (R-HSA-389546)	0.556	3
UniProt:Q6PI98 INO80C	1	INO80C [nucleoplasm] (R-HSA-5689605)	0.556	3
UniProt:Q8TBE0 BAHD1			0.556	3
UniProt:P23276 KEL	1	KEL [plasma membrane] (R-HSA-5694121)	0.556	3
UniProt:Q9NP61 ARFGAP3	1	ARFGAP3 [cytosol] (R-HSA-6807740)	0.556	3
UniProt:Q8NC51 SERB1			0.556	3
UniProt:P48730-2 CSNK1D			0.556	3
UniProt:P54253 ATX1			0.556	3
UniProt:Q16549 PCSK7			0.556	3
UniProt:P06756 ITGAV	3	ITGAV(31-1048) [specific granule membrane] (R-HSA-6806145) ITGAV(31-1048) [phagocytic vesicle] (R-HSA-1236773) ITGAV(31-1048) [plasma membrane] (R-HSA-210013)	0.556	3
UniProt:Q16586 SGCA			0.556	3
UniProt:Q99720-4 SIGMAR1			0.556	3
UniProt:P54252 ATXN3	2	ATXN3 [nucleoplasm] (R-HSA-6783285) ATXN3 [cytosol] (R-HSA-5625607)	0.556	3
UniProt:Q7Z614-3 SNX20			0.556	3
UniProt:P49184 DNASE1L1	2	DNASE1L1 [extracellular region] (R-HSA-6806320) DNASE1L1 [specific granule lumen] (R-HSA-6799554)	0.556	3
IntAct:EBI-25836632 CCSB			0.556	3
UniProt:Q13702-2 Q137022			0.556	3
UniProt:Q8TBJ4 PLPPR1	1	LPPR1 [plasma membrane] (R-HSA-6797625)	0.556	3
IntAct:EBI-25833683 CCSB			0.556	3
UniProt:Q6NTF9-3 Q6NTF93			0.556	3
UniProt:Q9NPC7 MYNN			0.556	3
UniProt:P12830 CDH1	8	CDH1(732-882) [plasma membrane] (R-HSA-3827997) CDH1(155-700) [plasma membrane] (R-HSA-2534244) CDH1(701-882) [extracellular region] (R-HSA-2534158) Ub-K,p-Y753,Y754-CDH1(155-882) [plasma membrane] (R-HSA-8877002) p-Y753,Y754-CDH1(155-882) [plasma membrane] (R-HSA-8876938) CDH1(155-750) [plasma membrane] (R-HSA-202940) CDH1(751-882) [plasma membrane] (R-HSA-202929) CDH1(155-882) [plasma membrane] (R-HSA-198003)	0.556	3
UniProt:P25490 YY1	1	YY1 [nucleoplasm] (R-HSA-4086091)	0.556	3
UniProt:P15036 ETS2	2	p-T72-ETS2 [nucleoplasm] (R-HSA-3132735) ETS2 [nucleoplasm] (R-HSA-1629811)	0.556	3
UniProt:P21246 PTN	1	PTN [extracellular region] (R-HSA-201500)	0.556	3
UniProt:O43463 SUV39H1	1	SUV39H1 [nucleoplasm] (R-HSA-427517)	0.556	3
UniProt:Q9NUD9 PIGV	1	PIGV [endoplasmic reticulum membrane] (R-HSA-162739)	0.556	3
UniProt:P54819 AK2	1	AK2 [mitochondrial intermembrane space] (R-HSA-57822)	0.556	3
UniProt:P0DTC3 3a	9	O-glycosyl 3a [virion membrane] (R-COV-9694395) O-glycosyl 3a [endocytic vesicle membrane] (R-COV-9694729) O-glycosyl 3a [endoplasmic reticulum-Golgi intermediate compartment membrane] (R-COV-9694386) GalNAc-O-3a [endoplasmic reticulum-Golgi intermediate compartment] (R-COV-9694658) 3a [endoplasmic reticulum-Golgi intermediate compartment] (R-COV-9694716) 3a [endoplasmic reticulum lumen] (R-COV-9694584) O-glycosyl 3a [endolysosome membrane] (R-COV-9754541) O-glycosyl 3a [plasma membrane] (R-COV-9694433) O-glycosyl 3a [extracellular region] (R-COV-9694387)	0.547	2
UniProt:P03372 ESR1	10	p-S118-ESR1 [nucleoplasm] (R-HSA-9024210) monoSUMO1-5K-ESR1 [nucleoplasm] (R-HSA-4090302) p-S167-ESR1 [nucleoplasm] (R-HSA-9841867) p-S167-ESR1 [cytosol] (R-HSA-9841254) Me260-ESR1 [cytosol] (R-HSA-9632177) PalmS-ESR1 [cytosol] (R-HSA-9020974) Me260-PalmS-ESR1 [plasma membrane] (R-HSA-9637788) PalmS-ESR1 [plasma membrane] (R-HSA-9021057) ESR1 [nucleoplasm] (R-HSA-446168) ESR1 [cytosol] (R-HSA-9020968)	0.527	3
UniProt:P02452 COL1A1	20	3x4Hyp-3Hyp-GlcGalHyl-COL1A1(23-1464) [endoplasmic reticulum lumen] (R-HSA-2023648) 3x4Hyp-GlcGalHyl-COL1A1(23-1464) [endoplasmic reticulum lumen] (R-HSA-2023667) GlcGalHyl-COL1A1(23-1464) [endoplasmic reticulum lumen] (R-HSA-2023604) GalHyl-COL1A1(23-1464) [endoplasmic reticulum lumen] (R-HSA-2022999) 3x4Hyp-3Hyp-GalHyl-COL1A1(23-1464) [endoplasmic reticulum lumen] (R-HSA-2023559) 3x4Hyp-GalHyl-COL1A1(23-1464) [endoplasmic reticulum lumen] (R-HSA-2023582) COL1A1(23-1464) [endoplasmic reticulum lumen] (R-HSA-1650777) 3x4Hyp-COL1A1(23-1464) [endoplasmic reticulum lumen] (R-HSA-1985659) 3x4Hyp-3Hyp-COL1A1(23-1464) [endoplasmic reticulum lumen] (R-HSA-2022446) 3x4Hyp-5Hyl-COL1A1(23-1464) [endoplasmic reticulum lumen] (R-HSA-2022951) 3x4Hyp-3Hyp-5Hyl-COL1A1(23-1464) [endoplasmic reticulum lumen] (R-HSA-2022954) 5Hyl-COL1A1(23-1464) [endoplasmic reticulum lumen] (R-HSA-1985663) Lysyl-pyrrolyl 5-hydroxylysyl collagen alpha-1(I) chain [extracellular region] (R-HSA-2396137) Lysyl-pyrrolyl 3,4-Hyp 5-Hyl collagen alpha-1(I) chain [extracellular region] (R-HSA-2396440) Lysyl-pyrrolyl 4-Hyp 5-Hyl collagen alpha-1(I) chain [extracellular region] (R-HSA-2396471) Lysyl-pyridinolyl 5-hydroxylysyl collagen alpha-1(I) chain [extracellular region] (R-HSA-2396464) Lysyl-pyridinolyl 4-Hyp 5-Hyl collagen alpha-1(I) chain [extracellular region] (R-HSA-2396068) Lysyl-pyridinolyl 3,4-Hyp 5-Hyl collagen alpha-1(I) chain [extracellular region] (R-HSA-2396388) Dehydrolysinonorlysyl 4-hydroxyprolyl collagen alpha-1(I) chain [extracellular region] (R-HSA-2396315) Dehydrolysinonorlysyl 4-Hyp 5-Gal-Hyl collagen alpha-1(I) chain [extracellular region] (R-HSA-2396441)	0.499	2

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