Cystatin A (CSTA)

This is an intracellular thiol proteinase inhibitor. Has an important role in desmosome-mediated cell-cell adhesion in the lower levels of the epidermis.

Cystatin A (CSTA) Related Areas

Enzyme>>Protease & Regulator>>Cysteine Protease & Regulator>>Cystatin>>Cystatin A/CSTA/Stefin A

Cancer>>Angiogenesis>>Cystatin>>Cystatin A/CSTA/Stefin A

Cystatin A (CSTA) Alternative Names

Cystatin A, CSTA, STF1, STFA, stefin A [Homo sapiens]

Cystatin A, Csta, cystatin-A, stefin-A [Mus musculus]

Summaries for Cystatin A (CSTA)

Entrez Gene summary for Cystatin A (CSTA):

The cystatin superfamily encompasses proteins that contain multiple cystatin-like sequences. Some of the members are active cysteine protease inhibitors, while others have lost or perhaps never acquired this inhibitory activity. There are three inhibitory families in the superfamily, including the type 1 cystatins (stefins), type 2 cystatins, and kininogens. This gene encodes a stefin that functions as a cysteine protease inhibitor, forming tight complexes with papain and the cathepsins B, H, and L. The protein is one of the precursor proteins of cornified cell envelope in keratinocytes and plays a role in epidermal development and maintenance. Stefins have been proposed as prognostic and diagnostic tools for cancer.

OMIM - description for Cystatin A (CSTA):

Cystatin A is a cysteine proteinase inhibitor that belongs to family 1 of the cystatin superfamily. It was originally derived from the cytosol of human polymorphonuclear granulocytes (Strauss et al., 1988) but has also been isolated from the spleen, liver, and epidermis. Cystatin A is identical to keratolinin, one of the precursor proteins of the cornified cell envelope of keratinocytes

Wikipedia summary for Cystatin A (CSTA):

Cystatin-A is a protein that in humans is encoded by the CSTA gene.The cystatin superfamily encompasses proteins that contain multiple cystatin-like sequences. Some of the members are active cysteine protease inhibitors, while others have lost or perhaps never acquired this inhibitory activity. There are three inhibitory families in the superfamily, including the type 1 cystatins (stefins), type 2 cystatins, and kininogens. This gene encodes a stefin that functions as a cysteine protease inhibitor, forming tight complexes with papain and the cathepsins B, H, and L. The protein is one of the precursor proteins of cornified cell envelope in keratinocytes and plays a role in epidermal development and maintenance. Stefins have been proposed as prognostic and diagnostic tools for cancer.

Human Cystatin A (CSTA) Protein General Information

 

• Protein names: Recommended name: Cystatin-A
• Sequence length: 98 AA.
• Sequence similarities: Belongs to the cystatin family.
• Subcellular location: Cytoplasm
• Tissue specificity: Expressed in the skin throughout the epidermis.
• Involvement in disease: Defects in CSTA are the cause of ichthyosis exfoliative autosomal recessive ichthyosis bullosa of Siemens-like (AREI) [MIM:607936]. A form of congenital exfoliative ichthyosis, sharing some features with ichthyosis bullosa of Siemens and annular epidermolytic ichthyosis. AREI presents shortly after birth as dry, scaly skin over most of the body with coarse peeling of non-erythematous skin on the palms and soles, which is exacerbated by excessive moisture and minor trauma. Electron microscopy analysis of skin biopsies, reveals mostly normal-appearing upper layers of the epidermis, but prominent intercellular edema of the basal and suprabasal cell layers with aggregates of tonofilaments in the basal keratinocytes.

General information above from UniProt

Function for Cystatin A (CSTA) Protein

UniProtKB:

This is an intracellular thiol proteinase inhibitor. Has an important role in desmosome-mediated cell-cell adhesion in the lower levels of the epidermis.

Genatlas:

• one of the precursor proteins of cornified cell envelope in keratinocytes and playing a role in epidermal development and maintenance
• cysteine cathepsin inhibitor, that may be a marker of increased cathepsin activity in metastasesn
• Cystatin A (CSTA) has antiapoptotic properties linked with neoplastic changes in squamous cell epithelium
• Cystatin A (CSTA) role for cystatin A in the normal physiology of the skin within the lower levels of the epidermis
• key role in basal to suprabasal keratinocyte adhesion

Phenotype Information for Cystatin A (CSTA)

• Gene/Locus: CSTA, STFA, STF1, AREI
• Phenotype: Exfoliative ichthyosis, autosomal recessive, ichthyosis bullosa of Siemens-like

Phenotype Information for Cystatin A (CSTA) from OMIM (Online Mendelian Inheritance in Man)