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University of Oulu

1. Huilaja, L. (Laura). Collagen XVII and pathomechanisms of junctional epidermolysis bullosa and gestational pemphigoid.

Degree: 2008, University of Oulu

Abstract Transmembrane collagen XVII (BP180) is a structural component of hemidesmosomes that connects the two layers of skin. Collagen XVII is associated with both autoimmune and inherited bullous skin diseases. Mutations in collagen XVII gene cause junctional epidermolysis bullosa, and in the diseases of the pemphigoid group autoantibodies target collagen XVII. In this work, collagen XVII was studied in both junctional epidermolysis bullosa and gestational pemphigoid. Two novel glycine substitution mutations were found in the largest collagenous domain of collagen XVII. Analysis of recombinantly produced mutated proteins showed that these novel mutations and previously described glycine substitution mutations decrease the thermal stability of collagen XVII ectodomain. In addition, these mutations were found to cause intracellular accumulation of the mutated proteins and affect the post-translational modifications of collagen XVII. Meanwhile, an in-frame deletion of nine amino acids had no effect on the thermal stability or secretion of the collagen XVII ectodomain. Gestational pemphigoid autoantigen collagen XVII has been mainly studied in the skin, and its expression and function during pregnancy are so far largely unknown. For the first time, collagen XVII was shown to be expressed by cytotrophoblasts of the first trimester human placenta and by cultured cytotrophoblasts. Transmigration assay of cytotrophoblasts indicated that collagen XVII promotes trophoblast invasion, and may thus have a role in placental formation. In addition, significant amounts of in vivo produced collagen XVII were found in the amniotic fluid throughout pregnancy. Collagen XVII expression was also observed in hemidesmosomes of amniotic membranes and in cells cultured from amniotic fluid. These findings suggest that collagen XVII could have a function, albeit so far unknown, during pregnancy.

Subjects/Keywords: BP180; basement membrane; blistering skin disease; collagen; epidermolysis bullosa; pemphigoid gestationis

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APA (6th Edition):

Huilaja, L. (. (2008). Collagen XVII and pathomechanisms of junctional epidermolysis bullosa and gestational pemphigoid. (Doctoral Dissertation). University of Oulu. Retrieved from http://urn.fi/urn:isbn:9789514287749

Chicago Manual of Style (16th Edition):

Huilaja, L (Laura). “Collagen XVII and pathomechanisms of junctional epidermolysis bullosa and gestational pemphigoid.” 2008. Doctoral Dissertation, University of Oulu. Accessed November 18, 2019. http://urn.fi/urn:isbn:9789514287749.

MLA Handbook (7th Edition):

Huilaja, L (Laura). “Collagen XVII and pathomechanisms of junctional epidermolysis bullosa and gestational pemphigoid.” 2008. Web. 18 Nov 2019.

Vancouver:

Huilaja L(. Collagen XVII and pathomechanisms of junctional epidermolysis bullosa and gestational pemphigoid. [Internet] [Doctoral dissertation]. University of Oulu; 2008. [cited 2019 Nov 18]. Available from: http://urn.fi/urn:isbn:9789514287749.

Council of Science Editors:

Huilaja L(. Collagen XVII and pathomechanisms of junctional epidermolysis bullosa and gestational pemphigoid. [Doctoral Dissertation]. University of Oulu; 2008. Available from: http://urn.fi/urn:isbn:9789514287749


Aristotle University Of Thessaloniki (AUTH); Αριστοτέλειο Πανεπιστήμιο Θεσσαλονίκης (ΑΠΘ)

2. Πατσατσή, Αικατερίνη. Ανίχνευση αυτοαντισωμάτων κατά των αντιγόνων BP180 και BP230 ως μέθοδος διάγνωσης και παρακολούθησης του πομφολυγώδους πεμφιγοειδούς.

Degree: 2008, Aristotle University Of Thessaloniki (AUTH); Αριστοτέλειο Πανεπιστήμιο Θεσσαλονίκης (ΑΠΘ)

Subjects/Keywords: Πομφολυγώδες πεμφιγοειδές; Bullous pemphigoid; BP180; BP230

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APA (6th Edition):

Πατσατσή, . . (2008). Ανίχνευση αυτοαντισωμάτων κατά των αντιγόνων BP180 και BP230 ως μέθοδος διάγνωσης και παρακολούθησης του πομφολυγώδους πεμφιγοειδούς. (Thesis). Aristotle University Of Thessaloniki (AUTH); Αριστοτέλειο Πανεπιστήμιο Θεσσαλονίκης (ΑΠΘ). Retrieved from http://hdl.handle.net/10442/hedi/23349

Note: this citation may be lacking information needed for this citation format:
Not specified: Masters Thesis or Doctoral Dissertation

Chicago Manual of Style (16th Edition):

Πατσατσή, Αικατερίνη. “Ανίχνευση αυτοαντισωμάτων κατά των αντιγόνων BP180 και BP230 ως μέθοδος διάγνωσης και παρακολούθησης του πομφολυγώδους πεμφιγοειδούς.” 2008. Thesis, Aristotle University Of Thessaloniki (AUTH); Αριστοτέλειο Πανεπιστήμιο Θεσσαλονίκης (ΑΠΘ). Accessed November 18, 2019. http://hdl.handle.net/10442/hedi/23349.

Note: this citation may be lacking information needed for this citation format:
Not specified: Masters Thesis or Doctoral Dissertation

MLA Handbook (7th Edition):

Πατσατσή, Αικατερίνη. “Ανίχνευση αυτοαντισωμάτων κατά των αντιγόνων BP180 και BP230 ως μέθοδος διάγνωσης και παρακολούθησης του πομφολυγώδους πεμφιγοειδούς.” 2008. Web. 18 Nov 2019.

Vancouver:

Πατσατσή . Ανίχνευση αυτοαντισωμάτων κατά των αντιγόνων BP180 και BP230 ως μέθοδος διάγνωσης και παρακολούθησης του πομφολυγώδους πεμφιγοειδούς. [Internet] [Thesis]. Aristotle University Of Thessaloniki (AUTH); Αριστοτέλειο Πανεπιστήμιο Θεσσαλονίκης (ΑΠΘ); 2008. [cited 2019 Nov 18]. Available from: http://hdl.handle.net/10442/hedi/23349.

Note: this citation may be lacking information needed for this citation format:
Not specified: Masters Thesis or Doctoral Dissertation

Council of Science Editors:

Πατσατσή . Ανίχνευση αυτοαντισωμάτων κατά των αντιγόνων BP180 και BP230 ως μέθοδος διάγνωσης και παρακολούθησης του πομφολυγώδους πεμφιγοειδούς. [Thesis]. Aristotle University Of Thessaloniki (AUTH); Αριστοτέλειο Πανεπιστήμιο Θεσσαλονίκης (ΑΠΘ); 2008. Available from: http://hdl.handle.net/10442/hedi/23349

Note: this citation may be lacking information needed for this citation format:
Not specified: Masters Thesis or Doctoral Dissertation


University of Oulu

3. Försti, A.-K. (Anna-Kaisa). Incidence, mortality, comorbidities, and treatment of bullous pemphigoid in Finland.

Degree: 2017, University of Oulu

Abstract Bullous pemphigoid (BP) is an autoimmune skin disease predominantly found in elderly people, which causes blistering of the skin and severe itching. The incidence of BP reported by previous studies has varied greatly between 0.05 and 42.8 per 1 million persons per year. Higher incidences have been reported in Western Europe and the USA, while countries around the Mediterranean have reported lower rates. However, the epidemiology of BP has not previously been studied in any Scandinavian country. The one-year mortality of BP is highly variable with estimates between 11% and 41% worldwide. As for comorbidities, the previous studies have shown that BP is associated with neurological disorders. The aim of this study was to investigate the incidence and mortality of BP in Finland, to assess the treatments used for BP, and the potential contribution of systemic glucocorticoid treatment to the high mortality rate found in BP patients. A further aim was to obtain more specific information about the neurological diseases associated with BP, and to clarify the less studied association with psychiatric disorders. For these purposes, we collected the records of all immunologically confirmed BP patients diagnosed in the Oulu University Hospital between 1985 and 2012, and, for a sub-study III, data for all patients diagnosed with BP in Finnish hospitals between 1987 and 2013. We found that the incidence of BP in Northern Finland has increased over the past two decades to approximately 27 new BP cases per 1 million persons per year. The one-year mortality of BP patients is 17%, and the standardized mortality ratio (SMR) is 7.6. Common comorbidities found in the sample of BP patients were: cardiovascular diseases (76%), neurodegenerative diseases (41%), skin conditions other than BP (37%) and type 2 diabetes (23%). Many neurodegenerative diseases of the central nervous system were associated with BP, as were many psychiatric disorders. The association was strongest between multiple sclerosis (MS) and BP, with MS patients having almost a 6-fold higher risk of BP than controls. The present study reports for the first time the incidence and mortality of BP in Finland, and provides new information about the association between BP and neurological and psychiatric disorders.

Tiivistelmä Rakkulainen pemfigoidi (josta jatkossa käytetään nimitystä pemfigoidi) on autoimmuunisairaus, joka esiintyy yleensä iäkkäillä, ja aiheuttaa ihon rakkulointia ja hankalaa kutinaa. Aiemmissa tutkimuksissa pemfigoidin ilmaantuvuus on vaihdellut 0,05:sta 42,8:aan tapaukseen miljoonaa ihmistä kohden vuodessa. Ilmaantuvuuden on havaittu olevan korkeampi Länsi-Euroopassa, kun taas Välimeren ympäristössä ilmaantuvuus on matalampi. Pemfigoidia sairastavien kuolleisuus vuoden kuluessa diagnoosista vaihtelee noin 11-41%:n välillä. Aiemmat tutkimukset ovat myös osoittaneet, että pemfigoidi liittyy neurologisiin sairauksiin. Pemfigoidin epidemiologiaa ei ole kuitenkaan tutkittu Suomessa tai muissa Pohjoismaissa. Tämän tutkimuksen tarkoituksena oli…

Advisors/Committee Members: Tasanen-Määttä, K. (Kaisa), Timonen, M. (Markku).

Subjects/Keywords: Finland; autoimmune bullous diseases; bullous pemphigoid; collagen XVII; epidemiology; incidence; mortality; neurological comorbidity; psychiatric comorbidity; skin diseases; standardized mortality ratio; Suomi; autoimmuunirakkulatauti; epidemiologia; ihosairaus; ilmaantuvuus; kollageeni XVII; kuolleisuus; neurologinen oheissairaus; pemfigoidi; psykiatrinen oheissairaus; rakkulainen pemfigoidi; vakioitu kuolleisuussuhde; BP180

Record DetailsSimilar RecordsGoogle PlusoneFacebookTwitterCiteULikeMendeleyreddit

APA · Chicago · MLA · Vancouver · CSE | Export to Zotero / EndNote / Reference Manager

APA (6th Edition):

Försti, A. -. (. (2017). Incidence, mortality, comorbidities, and treatment of bullous pemphigoid in Finland. (Doctoral Dissertation). University of Oulu. Retrieved from http://urn.fi/urn:isbn:9789526215310

Chicago Manual of Style (16th Edition):

Försti, A -K (Anna-Kaisa). “Incidence, mortality, comorbidities, and treatment of bullous pemphigoid in Finland.” 2017. Doctoral Dissertation, University of Oulu. Accessed November 18, 2019. http://urn.fi/urn:isbn:9789526215310.

MLA Handbook (7th Edition):

Försti, A -K (Anna-Kaisa). “Incidence, mortality, comorbidities, and treatment of bullous pemphigoid in Finland.” 2017. Web. 18 Nov 2019.

Vancouver:

Försti A-(. Incidence, mortality, comorbidities, and treatment of bullous pemphigoid in Finland. [Internet] [Doctoral dissertation]. University of Oulu; 2017. [cited 2019 Nov 18]. Available from: http://urn.fi/urn:isbn:9789526215310.

Council of Science Editors:

Försti A-(. Incidence, mortality, comorbidities, and treatment of bullous pemphigoid in Finland. [Doctoral Dissertation]. University of Oulu; 2017. Available from: http://urn.fi/urn:isbn:9789526215310

.