
formilglycin-nirman karnare enzyme

manavanmadhye 3p26.1 varr sthit formilglycin-generating enzyme (fge) heey endoplasmic reticulummadhye asalelya enzymeche naav aahe jeey systeinche formilglycinemadhye (eff. jee. aay.) rupantaran utprerit karate. eff. jee. eae. chey done mukhya varg aahet, aerobic aanee enarobic. eff. jee. eae. sulfate esterchya hasasasathi aavashyak asalelya sulphatases sakriy karate. sulfatseschi utprerak kriya sakriy thikanavaril formilglycin (kadhikadhi auxolanin mhanatat) avasheshanvar avalambun asate.
aerobic.
aerobic enzymechi rachana manvi sulphatage-sudharit ghatak 1 (samaph 1) chya januk utpadanat aadhalanaya jatil alfa/beeta topologieshy samaan aahe. aerobic eff. jee. eae. atyant sanrakshit sarvasanmatichya see. ex. pii. ex. aar. kramatil cysteine avasheshanche eff. jee. aay. madhye rupantar karate. asey karnyasathi, eff. jee. eae. mononuclear tambe vaparoon tyache lakshya "sakriy" karate. thurr pratham tambyashi bandhala jaato, jyamule oxygens thurr-tambe sankulachi pratikriyashilata vadhate. tyanantar thurr-tambe sankulatil cysteine avasheshanchya oxidationdware sakriyakaran purn kele jaate. yaa abhikriyechya swarupamule, eff. jee. eae. laa 'tambe-aadharit dhatoo-utsechak' mhanatat. 
avayaviy.
sarvat changlya prakare abhyasalela avayaviy eff. jee. eae. mhanje jivanu aii. tii. ess. bii., eka loh-sulfur samooh jyamadhye "clebasiela pneumonia" madhye asalele enzyme asate, jeey cysteine kinwa sereenche ektar eff. gly. madhye rupantarit karnyas saksham aahe, aerobic swarupapeksha vegalya yantranesah. aii. tii. ess. bii. ektar rupantarit karoo shakato, tarr serinavar systeinchya upasthitit tyachi kriya chaar patine vadhate. aii. tii. ess. bii. heey 'clostridium perfringence' madhye asalelya enzymesarkhech 48 takke aahe. donhi enzymesmadhye see. ex. 3see. ex. 2see. rachana asate jee radical ess-adenosyl methionine super familisathi adviteey aahe aanee ess-adenosyl methionineche vibhajan karnyasathi qamee karanyachi pratikriya vaparnyas saksham aahet. heey done enzymes avayaviy sulphatage paripakv enzymes navachya mothya gatamadhye yetat, jeey oxygencha vapar naan karata systeinche eff. jee. aay. madhye rupantar karnyas saksham asatat.
prathinanche kshetra.
aanvik jivashastrat, prathine utprerakdrushtya sakriy asso vaa naso, heey 'formilglycin-generating enzyme' (kadhikadhi formilglycin-generating sulphatage enzyme mhanoon bhashya kelele) heey eff. jee. eae. prathine kshetrache naav aahe. eff. jee. eae. chya procariotic aanee eucariotic homolog yaa donhimadhye atyant sanrakshit sakriy thikane asatat-jyat utsechak karyasathi aavashyak utprerak cysteine avasheshancha samavesh asato. aerobic jivanmadhye eff. jee. eae. utprerak sthalajaval sanrakshit avasheshandvare aanvik oxygenche sakriyakaran kele jaate asey manale jaate. utprerak cysteine avashesh thayol-systeinchya vinimayamadhye guntalele asatat jyamule eff. jee. aay. chey antim utpadan hote.
rog sthitee.
manavanmadhye, sumef1 madheel utparivartananmule eff. jee. eae. madhye dosh nirman hotat, jyamule sulfatsesche nuksan hote. parinami multiple sulphatage deficiency (emm. ess. dii.) navacha rog hoto, jyamadhye glycosaminoglycanes kinwa sulfolipids jamaa jhalyamule arbhakancha lavkar mrutyu houu shakato. haah rog pudhe navjat, usheera balant aanee kishore asha vegvegalya prakarat vibhagala jaau shakato, jyamadhye navjat haah sarvat gambhir asato. samanya lakshananmadhye ichithiosis, hypotonia, hadanchya vikrutee aanee ekoon sandnyanatmak ghat yancha samavesh hoto. 2017 madhye weidner aanee itar., sumef 1 abhivyakti aanee dirghakalin avarodhak fuffusiy rog (copd) vikasashi sambandh aadhalala. janewari 2020 paryant, jagbharat emm. ess. dii. chee 100 hoon adhik prakarane nondavali gelee. sumef1 sathee dnyat thurr aaheth aine-acetylgalectosamine-6-sulfate sulphatage (galnes), erilsulfatage aii (arsa), steroid sulphatage (ess. tii. ess.) aanee erilsulfatage eae (arse); cysteine asalele serve renu. eff. jee. eae. yaa cysteine gatala see-aii-formilglycinemadhye rupantarit karate. sumef 1 endoplasmic reticulum kinwa tyachya lumenmadhye aadhalate.