1. Gene
  2. PCSK6 - proprotein convertase subtilisin/kexin type 6 Gene

PCSK6 - proprotein convertase subtilisin/kexin type 6 Gene

Homo sapiens

Also known as SPC4; PACE4

Gene ID: 5046 | Gene type: protein coding

About PCSK6

Cytogenetic location: 15q26.3 Genomic coordinates (GRCh38): 15:101,303,933-101,489,707 (from NCBI)

This gene has 29 transcripts (splice variants), 196 orthologues and 9 paralogues. Biased expression in spleen (RPKM 31.3), liver (RPKM 30.7) and 12 other tissues.

Summary

This gene encodes a member of the subtilisin-like proprotein convertase family, which includes proteases that process protein and peptide precursors trafficking through regulated or constitutive branches of the secretory pathway. The encoded protein undergoes an initial autocatalytic processing event in the ER to generate a heterodimer which exits the ER and sorts to the trans-Golgi network where a second autocatalytic event takes place and the catalytic activity is acquired. The encoded protease is constitutively secreted into the extracellular matrix and expressed in many tissues, including neuroendocrine, liver, gut, and brain. This gene encodes one of the seven basic amino acid-specific members which cleave their substrates at single or paired basic residues. Some of its substrates include transforming growth factor beta related proteins, proalbumin, and von Willebrand factor. This gene is thought to play a role in tumor progression and left-right patterning. Alternatively spliced transcript variants encoding different isoforms have been identified. [provided by RefSeq, Feb 2014]

PCSK6 Products(7)

mRNA Protein Name
NM_001291309.2 NP_001278238.1 proprotein convertase subtilisin/kexin type 6 isoform i preproprotein
NM_002570.5 NP_002561.1 proprotein convertase subtilisin/kexin type 6 isoform PACE4-AI preproprotein
NM_138319.4 NP_612192.1 proprotein convertase subtilisin/kexin type 6 isoform PACE4-AII preproprotein
NM_138322.4 NP_612195.1 proprotein convertase subtilisin/kexin type 6 isoform PACE4B preproprotein
NM_138323.3 NP_612196.1 proprotein convertase subtilisin/kexin type 6 isoform PACE4CS preproprotein
NM_138324.3 NP_612197.1 proprotein convertase subtilisin/kexin type 6 isoform PACE4C preproprotein
NM_138325.4 NP_612198.2 proprotein convertase subtilisin/kexin type 6 isoform f preproprotein
Gene Ontology
  • Molecular Function
  • Biological Process
  • Cellular Component
Molecular Function GO Annotation Evidence Reference Source
enables endopeptidase activity IDA
IDA: Inferred from direct assay
9242664 GOA
enables heparin binding IDA
IDA: Inferred from direct assay
12535616 GOA
enables nerve growth factor binding IDA
IDA: Inferred from direct assay
8615794 GOA
enables protein binding IPI
IPI: Inferred from physical interaction
16433634 GOA
enables serine-type endopeptidase activity IDA
IDA: Inferred from direct assay
8218226 GOA
Biological Process GO Annotation Evidence Reference Source
involved in glycoprotein metabolic process IDA
IDA: Inferred from direct assay
8218226 GOA
involved in nerve growth factor production IDA
IDA: Inferred from direct assay
8615794 GOA
involved in peptide hormone processing IDA
IDA: Inferred from direct assay
9242664 GOA
involved in protein processing IDA
IDA: Inferred from direct assay
8218226 GOA
involved in secretion by cell IDA
IDA: Inferred from direct assay
8615794 GOA
Cellular Component GO Annotation Evidence Reference Source
located in cell surface IDA
IDA: Inferred from direct assay
12535616 GOA
located in collagen-containing extracellular matrix IDA
IDA: Inferred from direct assay
12535616 GOA
located in extracellular space IDA
IDA: Inferred from direct assay
8906861 GOA
EXP: Inferred from Experiment IDA: Inferred from direct assay IPI: Inferred from physical interaction IMP: Inferred from mutant phenotype IGI: Inferred from genetic interaction IEP: Inferred from expression pattern

PCSK6 Protein Structure

Peptidase_S8

Peptidase_S8: Subtilase family (199 - 486)

P_proprotein

P_proprotein: Proprotein convertase P-domain (538 - 628)

GF_recep_IV

GF_recep_IV: Growth factor receptor domain IV (693 - 810)

PLAC

PLAC: PLAC (protease and lacunin) domain (934 - 965)

  • 0
  • 200
  • 400
  • 600
  • 800
  • 968 a.a.
Protein Preferred Names Protein Names

proprotein convertase subtilisin/kexin type 6

paired basic amino acid cleaving enzyme 4

PCSK6 Protein-protein interaction Information

Type
Protein Name Protein ID Interactor Interactor Species Interactor ID Detection Method Reference
Intra
PCSK6 P29122 PCSK5 Homo sapiens Q92824-2
Validated Y2H
32296183
Intra
PCSK6 P29122 ZNF441 Homo sapiens Q8N8Z8
Validated Y2H
32296183
Cross: Cross-species interaction Intra: Intraspecies interaction

Recombinant PCSK6 Proteins

Cat. No. Product Name Accession Purity
HY-P700617 PCSK6 Protein, Human (His) P29122-1 (R860-G969) ≥95%
HY-P700621 PCSK6 Protein, Human (HEK293, hFc) P29122 (R860-G969) ≥95%

Related Diseases

Diseases Alias
Anthrax Disease

Anthrax

Ragpicker'S Disease

Black Baine

Malignant Edema

Malignant Pustule

Siberian Plague

Wool Sorter'S Disease

Gas Gangrene

Cutaneous Anthrax

Anthrax Infection

Splenic Fever

Melon Allergy

Cucumis Melo Fruit Allergy

Dyslexia
Arthrogryposis, Distal, Type 1c

DA1C

Distal Arthrogryposis Type 1c

Arthrogryposis, Distal, 1c

Hypobetalipoproteinemia, Familial, 2

Familial Hypobetalipoproteinemia 2

FHBL2

Hypolipidemia, Familial, Combined

Combined Familial Hypolipidemia

Combined Hypobetalipoproteinemia Familial

Hypobetalipoproteinemia, Familial, Type 2

Mitochondrial Complex Ii Deficiency

Isolated Mitochondrial Respiratory Chain Complex Ii Deficiency

Isolated Succinate-Coenzyme Q Reductase Deficiency

Isolated Succinate-Coq Reductase Deficiency

Isolated Succinate-Ubiquinone Reductase Deficiency

Mitochondrial Respiratory Chain Complex Ii Deficiency

Complex 2 Mitochondrial Respiratory Chain Deficiency

Succinate Coq Reductase Deficiency

Succinate Dehydrogenase Deficiency

Isolated Succinate Dehydrogenase Deficiency

Succinate-Coenzyme Q Reductase Deficiency

Malaria

Malaria, Susceptibility To

Malaria, Resistance To

Malaria, Cerebral

Cerebral Malaria

Malaria, Severe, Susceptibility To

Malaria, Severe, Resistance To

Malaria, Cerebral, Susceptibility To

Induced Malaria

Malaria, Vivax, Protection Against

Malaria, Severe

Malaria, Cerebral, Reduced Risk Of

Malaria, Protection Against

Resistance To Malaria Due To G6pd Deficiency

Malaria Due To G6pd Deficiency

Malarial Encephalitis

CM

Malaria Cerebral

Susceptibility To Malaria

Acute Pernicious Fever

Aestivo-Autumnal Fever

Aestivo Autumnal Malaria

Chagres Fever

Continued Malaria Fever

Estivo-Autumnal Fever

Estivo-Autumnal Malaria

Estivo-Autumnal Malarial Fever

Falciparum Fever

Malignant Tertian Fever

Malignant Tertian Malaria

Pernicious Intermittent Fever

Pernicious Malaria

Quotidian Malaria

Subtertian Fever

Subtertian Malaria Fever

Subtertian Malignant Tertian Malaria

Tropical Malaria

Algid Malaria

Bilious Haemoglobinuric Fever

Black Water Fever

Blackwater Fever

Malarial Blackwater Fever

Severe Malarial Falciparum

West African Fever

Malarial Haematinuria

Haemoglobinuric Fever

Haemoglobinuric Malaria

Severe Plasmodium Falciparum Malaria

Malarial Haemoglobinuria

Malarial Haematuria

Falciparum Malaria [Malignant Tertian]

Malaria Tropica

Malarial Shock

Chagres Virus Disease

Malignant Malaria

Mtm - [Malignant Tertian Malaria]

Tm -[Malignant Tertian Malaria]

Panama Fever

St - [Subtertian Malaria]

Malarial Quotidian

Benign Tertian Malaria

Tertian Ague

Vivax Fever

Plasmodium Vivax Malaria Nos

Btm - [Benign Tertian Malaria]

Bt - [Benign Tertian Malaria]

Vivax Malaria

Benign Tertian Vivax Malaria

Tertian Malaria

Quartan Malaria

Quartan Ague

Quartan Fever

Plasmodium Malariae Malaria Nos

Quartan Malarial

Malaria By Plasmodium Malariae

Malariae Malaria

Ovale Tertian Malaria

Plasmodium Ovale Fever

Malaria Fever By Plasmodium Ovale

Ovale Malaria

Malaria By Plasmodium Ovale

Malarial Ovale

Marsh Fever

Remittent Congestive Fever

Coastal Fever

Remittent Gastric Fever

Miasmatic Fever

Congestive Remittent Fever

Intermittent Fever

Jungle Fever

Paludism

Cameroon Fever

Ague

Corsican Fever

Intermittent Bilious Fever

Disease Due To Plasmodiidae

Malarial Fever

Plasmodiosis

Remittent Fever

Roman Fever

Malaria Fever Nos

Malaria Nos

Paludal Fever

Clinically Diagnosed Malaria

Clinically Diagnosed Malaria Without Parasitological Confirmation

Congestive Fever

Malarial Cachexia

Marsh Cachexia

Paludal Cachexia

Recurrent Malaria

Remittent Malaria

Diseases Alias
Waldenstroem'S Macroglobulinemia

Waldenstroem'S Macroglobulinemia

Waldenstroem'S Macroglobulinemia

Macroglobulinemia Of Waldenstrom

Lymphoplasmacytic Lymphoma With Igm Gammopathy

Lymphoplasmacytic Lymphoma

Waldenstroem'S Macroglobulinemia

Waldenstroem'S Macroglobulinemia

Macroglobulinemia Of Waldenstrom

Lymphoplasmacytic Lymphoma With Igm Gammopathy

Lymphoplasmacytic Lymphoma