Uloga izoformi plektina u organizaciji acetilkolinskih receptora i funkciji neuromuskularne veze

master's thesis

• Title (english): Plectin isoform specific role in acetylocholin receptor clustering and function of neuromuscular junction
• Author: Martina Žarak
• Mentor: Inga Marijanović (mentor)
• Committee member: Inga Marijanović (predsjednik povjerenstva)
• Committee member: Martina Šeruga Musić (član povjerenstva)
• Committee member: Ana Galov (član povjerenstva)
• Committee member: Maja Matulić (član povjerenstva)
• Granter: University of Zagreb; Faculty of Science; (Department of Biology); Zagreb
• Defense date and country: 2013-10-25, Croatia
• Scientific / art field, discipline and subdiscipline: NATURAL SCIENCES; Biology

Abstract

Plektin je citolinker protein koji ima važnu ulogu u funkciji stanice te je odgovoran za otpornost stanica na mehanički stres. Nedostatak plektina rezultira razvojem bulozne epidermiolize koja je u nekim slučajevima povezana s mišićnom distrofijom (EBD-MD) u kombinaciji s mijasteničnim sindromom (MyS), a koju karakterizira poremećaj u prijenosu signala na području neuromuskularne veze (NMV). Formiranje gustih nakupina AChR na postsinaptičkom dijelu NMV je ključno za učinkovit prijenos signala s motoričkih neurona na skeletne mišiće te je uloga plektina u ovom procesu predmet istraživanja ovog rada. Glavni cilj ovog istraživanja bio je utvrđivanje uloge izoformi plektina u organizaciji AChR te identifikacija specifičnih interacijskih partnera odgovornih za usmjeravanje plektina prema AChR kompleksu putem analize imunofluorescencije te in vitro testova vezivanja. Uloga plektina u organizaciji AChR je potvrđena, te je utvrđena specifičnost veze između izoforme plektina 1f i rapsina putem ABD plektina, kao i interakcija s intergrinom β1 te β-distroglikanom.

Abstract (english)

Plectin is a cytolinker protein important for vital cellular functions as well as mechanical resistance. Plectin deficiency results in epidermolysis bullosa simplex which in some patients is associated with muscular dystrophy (EBS-MD) and a severe myasthenic syndrome (MyS), characterized by impaired signal transmission at neuromuscular junctions (NMJs). For effective signal transmission from motor neurons to skeletal muscles, formation of dense AChR patches at the postsynaptic part of NMJs is of outmost importance and plectin’s involvement in this process was investigated in this thesis. The major goal of this project was the discovery of plectin isoform specific role in clustering of AChR and specific interaction partner(s) responsible for plectin’s targeting towards the AChR complex using immunofluorescence analysis and in vitro binding assays. The role of plectin in AChR clustering has been confirmed and specificity of the interaction between plectin 1f isoform and rapsyn via plectin’s ABD has been established, as well as with intergrin β1 and β-dystroglycan.

• Keywords: citolinker; rapsin; β-distroglikan; integrin β1; in vitro testovi vezivanja; imunofluorescencija
• Keywords (english): cytolinker; rapsyn; β-dystroglycan; integrin β1; in vitro binding assays; immunofluorescence
• Language: croatian
• URN:NBN: urn:nbn:hr:217:024235
• Study programme: Title: Molecular Biology; Study programme type: university; Study level: graduate; Academic / professional title: magistar/magistra molekularne biologije (magistar/magistra molekularne biologije)
• Type of resource: Text
• File origin: Born digital
• Access conditions: Access restricted to students and staff of home institution
• Created on: 2017-04-28 09:02:39