2018
DOI: 10.3390/ijms19113392
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Involvement of Aquaporins in the Pathogenesis, Diagnosis and Treatment of Sjögren’s Syndrome

Abstract: Sjögren’s syndrome (SS) is a chronic autoimmune disease characterized by lymphocytic infiltration of salivary and lacrimal glands resulting in diminished production of saliva and tears. The pathophysiology of SS has not yet been fully deciphered. Classically it has been postulated that sicca symptoms in SS patients are a double step process whereby lymphocytic infiltration of lacrimal and salivary glands (SG) is followed by epithelial cell destruction resulting in keratoconjunctivitis sicca and xerostomia. Rec… Show more

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Cited by 38 publications
(31 citation statements)
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“…It is also possible that reduced or absent tear secretion in patients with NGLY1 is an indirect result of defective AQP-dependent eye function. Patients with Sjogren's syndrome (OMIM-270150) exhibit decreased lacrimal and salivary gland secretion as a result of defective cellular trafficking of AQP5 (Soyfoo et al, 2018;Steinfeld et al, 2001;Groneberg et al, 2001). AQP1 gene therapy restored salivary and lacrimal gland fluid secretion in a mouse model of Sjogren's syndrome (Lai et al, 2016), again linking AQPs to lacrimal gland dysfunction.…”
Section: Discussionmentioning
confidence: 98%
“…It is also possible that reduced or absent tear secretion in patients with NGLY1 is an indirect result of defective AQP-dependent eye function. Patients with Sjogren's syndrome (OMIM-270150) exhibit decreased lacrimal and salivary gland secretion as a result of defective cellular trafficking of AQP5 (Soyfoo et al, 2018;Steinfeld et al, 2001;Groneberg et al, 2001). AQP1 gene therapy restored salivary and lacrimal gland fluid secretion in a mouse model of Sjogren's syndrome (Lai et al, 2016), again linking AQPs to lacrimal gland dysfunction.…”
Section: Discussionmentioning
confidence: 98%
“…AQP1 is expressed in myoepithelial and endothelial cells of mouse SG, but not in acinar and ductal cells [ 20 , 23 ]. AQP3, AQP4 and AQP8 are located at the basal region of acinar cells and in ductal cells [ 13 , 21 , 23 ]. AQP8 is also expressed by myoepithelial cells [ 24 ].…”
Section: Cellular Distribution Of Aqps In Adult Sg Subsectionmentioning
confidence: 99%
“…Noteworthy, however, is that the unorthodox AQP11 is glycerol permeable [ 11 ]. Several AQPs are expressed by the various SG cells [ 12 , 13 ], including AQP5 that plays an important role in the mechanism of saliva secretion [ 14 , 15 , 16 ]…”
Section: Introductionmentioning
confidence: 99%
“…Already in 1996, AQP5 gene therapy was considered as a possible medication for salivary glands impairment [Delporte et al, 1996]. New studies suggest AQP5 gene therapy as a potential treatment in Sjögren's syndrome patients, an autoimmune systemic disease with diminished saliva and tear production [Soyfoo et al, 2018].…”
Section: Discussionmentioning
confidence: 99%