hidradenitis suppurativa/protease

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Neutrophil-related host response in hidradenitis suppurativa: a pilot study in patients with inactive disease.

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Host-defence defects in hidradenitis suppurativa patients have been suspected, but not proven. Activated neutrophils can destroy the surrounding tissues by a release of reactive oxygen species and active proteases. Peripheral neutrophils from 15 female patients (mean age 46, range 27-57 years) in an

Acne inversa: evaluating antimicrobial peptides and proteins.

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BACKGROUND Acne inversa is a chronic, suppurative relapsing inflammatory skin disease that primarily affects the axillae, perineum and inframammary regions. Evidence suggests that the innate immune system is involved in the pathogenesis of acne inversa. OBJECTIVE To investigate the role of the

Two novel mutations of the nicastrin gene in Chinese patients with acne inversa.

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Acne inversa (AI, OMIM 142690), also called hidradenitis suppurativa, is a chronic, inflammatory, recurrent and debilitating skin follicular disease that usually presents after puberty with painful, deep-seated, inflamed lesions in the apocrine gland-bearing areas of the body, most commonly the

Impaired Notch-MKP-1 signalling in hidradenitis suppurativa: an approach to pathogenesis by evidence from translational biology.

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Recent findings in familial hidradenitis suppurativa (HS) demonstrated loss-of-function mutations of components of the γ-secretase (GS) complex leading to decreased protease cleaving activity, which may compromise canonical Notch signalling. Appropriate Notch signalling is of pivotal importance for

Two novel mutations of the NCSTN gene in Chinese familial acne inverse.

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BACKGROUND Acne inversa (AI; MIM 142690), or hidradenitis suppurativa (HS), is a type of autosomal-dominant genodermatosis caused by mutations in γ-secretase. The complex of γ-secretase is a transmembrane protease that catalyses the cleavage of a set of membrane proteins and is comprised of four

Expression of cathepsin L and its inhibitor hurpin in inflammatory and neoplastic skin diseases.

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Cathepsin (Cat) L is an important lysosomal proteinase involved in a variety of cellular functions including intracellular protein turnover, epidermal homeostasis and hair development. Hurpin (serpinB13) is a cross-class specific serine protease inhibitor of Cat L. We have analysed the expression

Essential requirement for nicastrin in marginal zone and B-1 B cell development.

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γ-secretase is an intramembrane protease complex that catalyzes the proteolytic cleavage of amyloid precursor protein and Notch. Impaired γ-secretase function is associated with the development of Alzheimer's disease and familial acne inversa in humans. In a forward genetic screen of mice with

Pyoderma gangrenosum and its syndromic forms: evidence for a link with autoinflammation.

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Pyoderma gangrenosum is a rare inflammatory neutrophilic dermatosis manifesting as painful ulcers with violaceous, undermined borders on the lower extremities. It may occur in the context of classic syndromes like PAPA (pyogenic arthritis, pyoderma gangrenosum and acne) and SAPHO (synovitis, acne,

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