
mannan-binding lectin

mannah-binding lectin (mbl), dheenini mannan-binding lectin ledhaa mannan-binding protein (mbp) anie kooda pilustaaru, idhi lectin maargam dwara opsonin gaa sahaja roganirodhaka saktiki keelaka paatra poeshistumdi.
nirmaanam.
mbl oka oligomeric nirmaanaanni (400-700 kda) kaligi undhi, idhi upa unitlatho nirminchabadindi, veetilo moodu oke vidhamaina peptide golusulu okkokkati 30 kda kaligi untaayi.
mbl aneka oligomeric roopaalanu yerparustundi, ayithe diamerlu mariyu trimmerlu opsoninga jeevasaastraparamgaa kriyaarahitamgaa unnayani mariyu complimentnu sakriyam cheyadaaniki kaneesam tetramer roopam avasaramani suuchanalu unnaayi.
janyuvulu mariyu polymorphisms.
maanava mbl2 janyuvu chromosome 10q11.2-q21 paie undhi. elukalaku rendu homolagus janyuvulu untaayi, conny maanavulalo vaatilo modatidi poyindi. kaaleyamlo mbl1 sudogen 1 (mbl1pii1) yokka takkuva sthaayi vyakteekarana kanugonabadindi. sudogen elukalu mariyu konni primatelalo mbla isopharmku samanamga unde 51-amaino assid proteinnu encode chestundi.
maanava mbl2 janyuvu yokka exon 1loe nirmaanaatmaka utparivartanalu, codan 52 (arg nundi sis, yugmavikalpam dii), codan 54 (gly nundi asp, yugmavikalpam bii) mariyu codan 57 (gly nundi gloo, yugmavikalpam sii) vadda, protein yokka collogenous nirmaanaaniki antaraayam kaliginchadam dwara kriyaatmaka seeram mbl sthaayini swatantramgaa taggistaayi. inca, mbl2 janyuvu yokka prothsaahaka praantamloo-550 (hetch/ell polymorphism),-221 (ex/vai polymorphism) mariyu-427,-349,-336, dell (-324 nundi-329),-70 mariyu + 4 (pii/kyuu polymorphisms) sthaanaalalo aneka nucleotide pratyaamnaayaalu mbl seeram gaadatanu prabhaavitam chestaayi. balamaina anusandhaana asamatulyatalo unna nirmaanaatmaka utparivartanala tarachudanam mariyu prothsaahaka bahuroopatalu rendoo jaathi samuuhaala madhya maarutuu untaayi, phalitamgaa edu pradhana haplotypes erpadataayih hypa, lyca, laipa, lxpa, lipe, like mariyu hype. eee haplotype la pampinheelo thedaalu mbl seeram sthaayilalo jaatyaantara vaividhyaalaku pradhana kaaranam. hypa mariyu lyca rendoo adhika utpatti chese haplotypes, laipa intermediate utpatti chese haplotype mariyu lxpa takkuva utpatti chese haplotype, ayithe lipe, like mariyu hype lopabhuishta haplotypes, ivi teevramaina mbl lopaniki kaaranamavutaayi.
praimaetla parinaamam antataa mbl2 mariyu mbl1pii1 janyuvulu rendoo padepade debbatinnaayi. collagen lanty praantamlooni glycine avasheshaalalo utparivartana dwara rendodi chivariki nissabdam cheyabadindi. maanavulalo mbl2 roopaantara yugma vikalpaalaku kaaranamayye adhe paramaanu yantraamgaala dwara parinaama samayamlo idhi empika cheyabadindi, idhi takkuva utpatti chese mbl janyuvulaku parinaamaatmaka empikanu suuchistumdi.
anuvaadaanantara maarpulu.
eluka hepatocyteslo, mbl kathinamaina endoplasmic reticulumlo samslaeshanha cheyabadutundi. golgiiloo, idhi rendu vibhina anuvaadaanantara maarpulaku lonavutundi mariyu adhika paramaanu baruvu gala multimeric complexelalo sameekarinchabadutundi. eee maarpulu mblnu koddigaa vibhina paramaanu dravyaraasulu mariyu paie yokka bahula roopaalalo 5.7 nundi 6.2 varaku utpatti chestaayi. proteolytic cheelika kooda 20-aaa enn-terminal signal peptidenu tolaginchadaaniki daariteesindi, mariyu hydroxylation mariyu glycosylation kooda kanugonabaddayi. konni systeine avasheshaalanu dehydroalaninga maarchavacchu.
function.
mbl sii-rakam lectin suupar familyloni collectinla taragatiki chendinadi, dheeni paniteeru prii-emmune hostlo modati varusa rakshanalo namuunaa gurtimpugaa kanipistundi. bacteria, vairaslu, protojova mariyu sileendhraalatoo sahaa pedda sankhyalo vyadhikaaraka suukshmajeevula uparitalampai kanipinche carbohydrate namuunaalanu mbl gurtistundi. suukshmajeeviki mbl bandhinchadam valla paripuurakaramaina vyavastha yokka lectin maargam kriyaaseelakam avutundi.
mbl yokka maroka mukhyamaina pani emitante, eee anuvu senescent mariyu apoptotic kanaalanu bandhistundi mariyu mottam, chekkuchedarani apoptotic kanaalanu, alaage phagocytela dwara kana sidhilaalanu aavarimpajestundi.
kriyaaseelata.
paripuurakaramaina vyavasthanu moodu maargaala dwara sakriyam cheyavachuhu shaastreeya maargam, pratyaamnaaya maargam mariyu lectin maargam. iteevala kanugonna lectin maargam sakriyam cheyabadina oka maargam mannos-binding lectin protein dwara. mbl aneka vyadhikaaraka kaarakaala uparitalaalapai kanipinche carbohydratelatho (nirdishtamgaa cheppalante, dii-mannos mariyu ell-fucose avasheshaalu) bandhistundi.
udaaharanaku, mbl dheeniki kattubadi unnatlu choopabadindihi
complexes.
raktamlo mbl anedi masp (mbl-sambandhita serin protease) anie piluvabade serin proteejtho (kattubadi) sanklishtamgaa untundi. moodu maaspulu unnaih masp-1, masp-2 mariyu masp-3, ivi protease domainlanu kaligi untaayi. protease domainlu laeni mariyu masps yokka niyantrana anuvulugaa bhaavinche smap (mapp 19 anie kooda pilustaaru) mariyu mapp 44 kooda unnaayi. masps kooda phicolinlatho sanklishtatalanu yerparustaayi, ivi mbl maadirigaane panicheestaayi mariyu nirmanatmakamga untaayi, minaha phicolinlu mbl laaga kakunda fibrinogen-vanti domainla dwara tama lakshyalanu gurtistaayi.
mbl daani lakshyaaniki bandhinchinappudu paripuurakaramaina vyavasthanu sakriyam cheyadaaniki (udaaharanaku, bacteria uparitalampai mannos), masp protein rakta protein sii4nii sii4eah mariyu sii4biga cheelchadaaniki panichestundi. c4b sakalaalu appudu bacteria uparitalamtho bandhinchi, c3-convertase erpadataaniki praarambhinchavacchu.
c3-convertase dwara utprerakam cheyabadina tadupari puuraka cascade pora daadi samudaayaanni srushtistundi, idhi apoptotic mariyu necrotic kanaala sandarbhamlo vyadhikaaraka yokka lysisto paatu sweeya maarpuku kaaranamavutundi.
mbl/masp-1 complexes kooda thrombin lanty kaaryaacharananu kaligi untundi (raktam gaddakattadanni praarambhinchadaaniki thrombin gaddalu fibrin). janyuparamgaa mbl ledhaa masp-1/3 (conny masp-2/smap kaadu) laeni elukalu prayogaatmaka gaayam namoonaalalo sudeergha raktasraavam samayanni kaligi untaayi, ayithe sariiraaniki avamanakaram lekapothe elukalu saadhaaranamainavigaa kanipistaayi.
clinical praamukhyata.
idhi sankramanaku pratispandanagaa kaaleyamlo utpatti avutundi, mariyu teevramaina dasha proteins anie piluvabade aneka itara karakalalo bhaagam. itara avayavaalalo vyakteekarana mariyu paniteeru kooda suuchimchabaddaayi.
exon 1 yokka moodu nirmaanaatmaka bahuroopatalu meningococcal vyaadhitho sahaa vividha saadhaarana antuvyaadhulaku gurayye avakaasam kaligistaayani nivedinchabadindi. ayithe, mengingococcal vyaadhiki sambandhinchi eee vaividhyaala valla etuvanti haanikaramaina prabhavam undadani soochinche aadharalu samarpinchabaddaayi. mbl lopam maanavulalo chaalaa saadhaaranam, sumaru 10 saatam mandhi vyaktulu eee lopaanni kaligi unnaaru.

