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Protein Page:
SPINK5 (human)

Overview
SPINK5 Serine protease inhibitor, probably important for the anti-inflammatory and/or antimicrobial protection of mucous epithelia. Contribute to the integrity and protective barrier function of the skin by regulating the activity of defense- activating and desquamation-involved proteases. Inhibits KLK5, it's major target, in a pH-dependent manner. Inhibits KLK7, KLK14 CASP14, and trypsin. Defects in SPINK5 are the cause of Netherton syndrome (NETH). NETH is an autosomal recessive congenital ichthyosis associated with hair shaft abnormalities and anomalies of the immune system. Typical features are ichthyosis linearis circumflexa, ichthyosiform erythroderma, trichorrhexis invaginata (bamboo hair), atopic dermatitis, and hayfever. High postnatal mortality is due to failure to thrive, infections and hypernatremic dehydration. 3 isoforms of the human protein are produced by alternative splicing. Note: This description may include information from UniProtKB.
Protein type: Secreted; Secreted, signal peptide; Inhibitor protein
Cellular Component: endoplasmic reticulum membrane; endoplasmic reticulum; perinuclear region of cytoplasm; cytoplasm; extracellular region; cell cortex; cytosol
Molecular Function: serine-type endopeptidase inhibitor activity
Biological Process: negative regulation of proteolysis; regulation of cell adhesion; negative regulation of angiogenesis; extracellular matrix organization and biogenesis; epithelial cell differentiation; negative regulation of immune response; hair cell differentiation; epidermal cell differentiation; regulation of T cell differentiation; anagen
Reference #:  Q9NQ38 (UniProtKB)
Alt. Names/Synonyms: DKFZp686K19184; FLJ21544; FLJ97536; FLJ97596; FLJ99794; Hemofiltrate peptide HF6478; Hemofiltrate peptide HF7665; ISK5; LEKTI; LETKI; Lympho-epithelial Kazal-type-related inhibitor; lymphoepithelial Kazal-type-related inhibitor; NETS; NS; serine peptidase inhibitor, Kazal type 5; Serine protease inhibitor Kazal-type 5; serine protease inhibitor, Kazal type 5; SPINK5; VAKTI
Gene Symbols: SPINK5
Molecular weight: 120,714 Da
Basal Isoelectric point: 8.45  Predict pI for various phosphorylation states
Select Structure to View Below

SPINK5

Protein Structure Not Found.


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Modification Sites and Domains  

Modification Sites in Parent Protein, Orthologs, and Isoforms  
 

Show Multiple Sequence Alignment


 SS 

SS: The number of records in which this modification site was determined using site-specific methods. SS methods include amino acid sequencing, site-directed mutagenesis, modification site-specific antibodies, specific MS strategies, etc.


 MS 

MS: The number of records in which this modification site was assigned using ONLY proteomic discovery-mode mass spectrometry.


       human

 
0 1 N837 SNTGERSNTGERSND
  mouse

 
S764 RNTETNKSDKEDKCH
  rat

 
S814-p RNTGANKsNKKQGNS
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