Serpinh1 serine (or cysteine) peptidase inhibitor, clade H, member 1 [Mus musculus] - Gene

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Summary

Official Symbol: Serpinh1provided by MGI
Official Full Name: serine (or cysteine) peptidase inhibitor, clade H, member 1provided by MGI
Primary source: MGI:88283
See related: Ensembl:ENSMUSG00000070436
Gene type: protein coding
RefSeq status: VALIDATED
Organism: Mus musculus
Lineage: Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; Eutheria; Euarchontoglires; Glires; Rodentia; Sciurognathi; Muroidea; Muridae; Murinae; Mus; Mus
Also known as: J6; Cbp1; Cbp2; gp46; Hsp47; BERF-1; Serpinh2; colligin

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Genomic context

Location :: 7 E2; 7

Sequence :: Chromosome: 7; NC_000073.6 (99345375..99353239, complement)

See Serpinh1 in Epigenomics, MapViewer

Chromosome 7 - NC_000073.6 Genomic Context describing neighboring genes

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Genomic regions, transcripts, and products

Genomic Sequence

Go to nucleotideGraphics FASTA GenBank

Go to reference sequence details

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Bibliography

GeneRIFs: Gene References Into Functions What's a GeneRIF?

overexpression of HSP47 in keloid fibroblast cells could induce excessive collagen accumulation by enhancing collagen synthesis, which not only presents a possible mechanism of keloid formation
Title: Effect of heat shock protein 47 on collagen synthesis of keloid in vivo.
alpha-smooth-muscle-actin-positive myofibroblast of bleomycin-induced pulmonary fibrosis cells may synthesize procollagen in the fibrotic process of bleomycin-treated lungs through upregulation of HSP47 mRNA and play an important role in fibrogenesis.
Title: Localization of HSP47 mRNA in murine bleomycin-induced pulmonary fibrosis.
the HSP47-specific siRNA inhibited expression of HSP47 at the level of mRNA and protein, and furthermore, adenovirus-mediated transfer of siRNA against HSP47 inhibited the expression of type I collagen and formation of scar tissue in animal mod
Title: Adenovirus-mediated RNA interference against collagen-specific molecular chaperone 47-KDa heat shock protein suppresses scar formation on mouse wounds.
type IV collagen in dilated endoplasmic reticulum leads to apoptosis in Hsp47-knockout mouse embryos via induction of CHOP
Title: Accumulation of type IV collagen in dilated ER leads to apoptosis in Hsp47-knockout mouse embryos via induction of CHOP.
HSP47 localized in alpha-smooth muscle actin-positive myofibroblasts, F4/80 negative, surfactant protein-A-positive type II pneumocytes, and F4/80-positive macrophages. Cells may play role in fibrotic process through upregulation of HSP47.
Title: Increased expression of collagen-binding heat shock protein 47 in murine bleomycin-induced pneumopathy.
analysis of the client recognition mechanism of HSP47
Title: Specific recognition of the collagen triple helix by chaperone HSP47: minimal structural requirement and spatial molecular orientation.
Hsp47 is required for correct folding and prevention of aggregation of type I collagen in the ER.
Title: Type I collagen in Hsp47-null cells is aggregated in endoplasmic reticulum and deficient in N-propeptide processing and fibrillogenesis.
analysis of recognition of the collagen triple helix by chaperone HSP47
Title: Specific recognition of the collagen triple helix by chaperone HSP47. II. The HSP47-binding structural motif in collagens and related proteins.
Results indicate that Hsp47 is required for the molecular maturation of type IV collagen and suggest that misfolded type IV collagen causes abnormal morphology of embryoid bodies.
Title: Insufficient folding of type IV collagen and formation of abnormal basement membrane-like structure in embryoid bodies derived from Hsp47-null embryonic stem cells.

Submit: New GeneRIF Correction

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Alleles

Alleles of this type are documented at Mouse Genome Informatics (MGI)

Targeted (knock-out) (1) 1 citation

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Function	Evidence Code	Pubs
protein binding	IPI Inferred from Physical Interaction more info	PubMed
serine-type endopeptidase inhibitor activity	IBA Inferred from Biological aspect of Ancestor more info
unfolded protein binding	IDA Inferred from Direct Assay more info	PubMed

Process	Evidence Code	Pubs
collagen biosynthetic process	IMP Inferred from Mutant Phenotype more info	PubMed
collagen fibril organization	IMP Inferred from Mutant Phenotype more info	PubMed
negative regulation of endopeptidase activity	IBA Inferred from Biological aspect of Ancestor more info
protein maturation	IMP Inferred from Mutant Phenotype more info	PubMed
regulation of proteolysis	IBA Inferred from Biological aspect of Ancestor more info
response to stress	IEA Inferred from Electronic Annotation more info

Component	Evidence Code	Pubs
cytoplasm	IDA Inferred from Direct Assay more info	PubMed
endoplasmic reticulum	ISO Inferred from Sequence Orthology more info
endoplasmic reticulum-Golgi intermediate compartment	ISO Inferred from Sequence Orthology more info

General protein information

Preferred Names: serpin H1

Names: serpin H1; collagen-binding protein; 47 kDa heat shock protein; collagen binding protein 1; serine protease inhibitor J6; beta enolase repressor factor 1; serine (or cysteine) proteinase inhibitor, clade H, member 1; serine (or cysteine) proteinase inhibitor, clade H, member 2; serine (or cysteine) proteinase inhibitor, clade H (heat shock protein 47), member 1; serine (or cysteine) proteinase inhibitor, clade H (heat shock protein 47), member 2

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NCBI Reference Sequences (RefSeq)

RefSeqs maintained independently of Annotated Genomes

These reference sequences exist independently of genome builds. Explain

These reference sequences are curated independently of the genome annotation cycle, so their versions may not match the RefSeq versions in the current genome build. Identify version mismatches by comparing the version of the RefSeq in this section to the one reported in Genomic regions, transcripts, and products above.

mRNA and Protein(s)

NM_001111043.1 → NP_001104513.1 serpin H1 precursor

Status: VALIDATED

Source sequence(s)

AV300388, BB686988, BC085143, CA751137

Consensus CDS

CCDS21480.1

UniProtKB/Swiss-Prot

P19324

Related

ENSMUSP00000091706, ENSMUST00000094154

Conserved Domains (1) summary

cl00137
Location:40 – 405
Blast Score: 1662

SERPIN; SERine Proteinase INhibitors (serpins) exhibit conformational polymorphism shifting from native to cleaved, latent, delta, or polymorphic forms. Many serpins, such as antitrypsin and antichymotrypsin, function as serine protease inhibitors which regulate ...
NM_001111044.1 → NP_001104514.1 serpin H1 precursor

Status: VALIDATED

Source sequence(s)

AK077937, AV300388, BB686988, CA751137

Consensus CDS

CCDS21480.1

UniProtKB/Swiss-Prot

P19324

Conserved Domains (1) summary

cl00137
Location:40 – 405
Blast Score: 1662

SERPIN; SERine Proteinase INhibitors (serpins) exhibit conformational polymorphism shifting from native to cleaved, latent, delta, or polymorphic forms. Many serpins, such as antitrypsin and antichymotrypsin, function as serine protease inhibitors which regulate ...
NM_009825.2 → NP_033955.2 serpin H1 precursor

Status: VALIDATED

Source sequence(s)

AK031457, AV300388, BB686988, CA751137

Consensus CDS

CCDS21480.1

UniProtKB/Swiss-Prot

P19324

Related

ENSMUSP00000126390, ENSMUST00000169437

Conserved Domains (1) summary

cl00137
Location:40 – 405
Blast Score: 1662

SERPIN; SERine Proteinase INhibitors (serpins) exhibit conformational polymorphism shifting from native to cleaved, latent, delta, or polymorphic forms. Many serpins, such as antitrypsin and antichymotrypsin, function as serine protease inhibitors which regulate ...

RefSeqs of Annotated Genomes: Build 38.1

The following sections contain reference sequences that belong to a specific genome build. Explain

Reference GRCm38 C57BL/6J

Genomic

NC_000073.6 Reference GRCm38 C57BL/6J

Range

99345375..99353239, complement

Download

GenBank, FASTA, Sequence Viewer (Graphics)

Alternate Mm_Celera

Genomic

AC_000029.1 Alternate Mm_Celera

Range

99670192..99678104, complement

Download

GenBank, FASTA, Sequence Viewer (Graphics)

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Related Sequences

Nucleotide		Protein	Strain
Heading	Accession and Version	Protein	Strain
genomic	AC158748.9 (111662..119382)	None
genomic	CH466531.1	EDL16377.1	mixed
		EDL16378.1	mixed
genomic	D12907.2	BAA02298.1	BALB/c
mRNA	AK029080.1	BAC26283.1	C57BL/6J
mRNA	AK031457.1	BAC27413.1	C57BL/6J
mRNA	AK076474.1	BAC36360.1	C57BL/6J
mRNA	AK077660.1	BAC36935.1	C57BL/6J
mRNA	AK077937.1	BAC37075.1	C57BL/6J
mRNA	AK077992.1	None	C57BL/6J
mRNA	AK079460.1	BAC37652.1	C57BL/6J
mRNA	AK086519.1	BAC39683.1	C57BL/6J
mRNA	AK086523.1	BAC39684.1	C57BL/6J
mRNA	AK089993.1	BAC41032.1
mRNA	AK090103.1	BAC41093.1
mRNA	AK159697.1	BAE35297.1	C57BL/6J
mRNA	AK159998.1	BAE35546.1	C57BL/6J
mRNA	AK166000.1	BAE38510.1
mRNA	AK167403.1	BAE39492.1	C57BL/6J
mRNA	AK168442.1	BAE40347.1	C57BL/6J
mRNA	AK168870.1	BAE40689.1	C57BL/6J
mRNA	AK169422.1	None	C57BL/6J
mRNA	AK205936.1	None
mRNA	AK206725.1	None
mRNA	AK209349.1	None
mRNA	AK211972.1	None
mRNA	AK219072.1	None
mRNA	AV300388.1	None	C57BL/6J
mRNA	BB686988.1	None	C57BL/6J
mRNA	BC085143.1	AAH85143.1	C57BL/6
mRNA	CA751137.1	None	C57BL/6
mRNA	J05609.1	AAA03200.1
mRNA	X60676.1	CAA43091.1	BALB/c

Protein Accession	Links
Protein Accession	GenPept Link	UniProtKB Link
P19324.3	GenPept	UniProtKB/Swiss-Prot:P19324
Q3TJK3	GenPept	UniProtKB/TrEMBL:Q3TJK3
Q3TMD2	GenPept	UniProtKB/TrEMBL:Q3TMD2
Q3TWG9	GenPept	UniProtKB/TrEMBL:Q3TWG9
Q8BV87	GenPept	UniProtKB/TrEMBL:Q8BV87
Q8BVU9	GenPept	UniProtKB/TrEMBL:Q8BVU9