
pee. aiss. aim. bee. 8

proteojom subunit beeta type-8 jise 20aiss proteojom subunit beeta-5aaee key roop mein jaanaa jaataa haye, ack protein haye jise manushyon mein "pee. aiss. aim. bee. 8" jeane dwara kootbaddh kiya jaataa haye.
yeh protein 17 aavashyak upa-ikaaiyon (alfa upa-ikaaiyaan 1-7, gathanaatmak beeta upa-ikaaiyaan 1-7, aur beeta1आee, beeta2आee, beeta5आee sahit prerak upa-ikaaiyaan) mein seey ack haye joe 20aiss protesome complexes kii poori assembly mein yogdan detaa haye. vishesh roop seey, anya beeta upa-ikaaiyon key saath proteojom upa-ikai beeta prakaar-5, dou heptameric rings mein ikattha hotaa haye aur baad mein substrate ksharan key liye ack proteolytic kaksh. iss protein mein "chimotrypsin jaisi" gatividhi hoti haye aur yeh peptide key badey hydrophobic avasheshon key baad alag honey mein saksham haye. eukariotic proteojom ney protein kii gunavatta niyantran uddeshya key liye kshatigrast protein yaa gatishil javic prakriyaon key liye pramukh niyaamak protein ghatakon sahit apakshayi protein koo maanyata dee. jaba koshikaaen interferon-γ key upchaar key tahat hoti hain tou sanrachanaatmak upa-ikai beeta1, beeta2, aur beeta5 (vyavasthit namkaran) koo unake prerak samkaksh beeta1i, 2i, aur 5i dwara pratisthaapit kiya jaa sakata haye. parinami protesome complexes tathaakathit immunoprotesome bunn jaataa haye. sanshodhit protesome parisar, immunoprotesome kaa ack aavashyak kaary, kaee aim. aich. see. varg-aaee pratibandhit tii koshika epitope kaa prasanskaran haye.
sanrachana.
jeane.
yeh jeane proteojom bee-type parivaar key ack sadasya koo kootbaddh kartaa haye, jise tii1bee parivaar key roop mein bhee jaanaa jaataa haye, joe ack 20aiss core beeta upa-ikai haye. yeh jeane aim. aich. see. (pramukh ooti-sangatata parisar) key varg II kshetra mein sthit haye. iss jeane kii abhivyakti gaama interferon dwara prerit hoti haye aur yeh jeane utpaad immunoprotesome mein utprerak upghatak 3 (protesome beeta 5 upghatak) kii jagah letaa haye. ack paripakva upa-ikai utpann karane key liye proteolytic prasanskaran kii aavashyakta hoti haye. dou vaikalpik pratilekhon koo dou samroopon koo kootbaddh karane key liye pahachaana gayaa haye; donon samroopon koo ack hii paripakva upghatak dene key liye sansaadhit kiya jaataa haye.
maanav pee. aiss. aim. bee. 8 jeane mein 7 exon hotey hain aur gunasutra baind 6p21.3 parr sthit hotey hain.
protein sanrachana.
maanav protein proteojom subunit beeta prakaar-8 aakar mein 23 kda haye aur 204 emino acid seey banaa haye. iss protein kaa ganana kiya gayaa saiddhaantik paaee 7.59 haye.
jatil sabhaa.
protesome ack bahu-utprerak protein parisar haye jisamen ack uchch krambaddh 20s mool sanrachana hoti haye. yeh bairal key aakar kii mool sanrachana 28 gair-samaan upa-ikaaiyon key 4 akshiya roop seey dher kiye gaye valayon seey bunny haih dou anta valay pratyek 7 alfa upa-ikaaiyon dwara banaae jaate hain, aur dou kendriya valay pratyek 7 beeta upa-ikaaiyon dwara banaae jaate hain. teen beeta upa-ikaaiyon (beeta1, beeta2, beeta5) mein seey pratyek mein ack proteolytic sakriya site hoti haye aur isaki alag-alag substrate praathamiktaaen hoti hain. proteojoms poore eukariotic koshikaon mein uchch sandrata parr vitarit kiye jaate hain aur ack gair-lysosomal maarg mein ack aeye. tii. pee./ubquitin-nirbhar prakriya mein peptides koo alag karate hain.
kaary.
protein kaary isaki tritiyak sanrachana aur sahayogi bhagidaron key saath isaki baatchit dwara samarthit hain. 20s proteojom kii 28 upa-ikaaiyon mein seey ack key roop mein, protein proteojom upa-ikai beeta prakaar-2 substrate ksharan key liye ack proteolytic vatavaran banaane mein yogdan detaa haye. prithak 20s proteojom parisar kii chrystal sanrachanaon key sakshya pradarshit karate hain kii beeta upa-ikaaiyon key dou valay ack proteolytic kaksh banaate hain aur kaksh key bheetar proteolysis key apane sabhi sakriya sthalon koo banaae rakhate hain. saath hii, alfa upa-ikaaiyon key valay proteolytic kaksh mein pravesh karane vaale substrate key liye pravesh dwaar banaate hain. ack nishkriya 20s proteojom parisar mein, aantarik proteolytic kaksh mein pravesh dwaar vishisht alfa-subunit kii aine-terminal poonchh dwara sanrakshit hotaa haye. yeh advitiya sanrachana abhikalpana proteolytic sakriya sthalon aur protein substrate key beach yaadrichhik muthbhed koo rokti haye, joe protein ksharan koo ack achchi tarah seey viniyamit prakriya banaati haye. 20s proteojom complexes, apane aap mein, aamtaur parr karyatmak roop seey nishkriya hotaa haye. 20s core kann (cp) kii proteolytic kshamata taba sakriya kii jaa sakati haye jaba cp alfa ring key ack yaa donon taraf ack yaa dou niyaamak kanon (rp) key saath sambaddh hotaa haye. inn niyaamak kanon mein 19aiss proteojom parisar, 11aiss proteojom parisar aadi shamil hain. see. pee.-aar. pee. sangh key baad, kuchh alfa upa-ikaaiyon kii pushti badal jaayegi aur isake parinamswarup substrate pravesh dwaar khul jaayega. aar. pee. aiss. key alawa, 20 key proteojom koo anya halke rasayanik upchaaron dwara bhee prabhaavi dhang seey sakriya kiya jaa sakata haye, jaise kii sodium dodecilsulfate (aiss. dee. aiss.) yaa aine. pee.-14 key nimn star key sampark mein aanaa.
20s proteojom subunit beeta-5i (vyavasthit namkaran) koo mool roop seey 276 emino acid key saath ack agradoot key roop mein vyakt kiya jaataa haye. peptide aine-terminal parr 72 emino acid kaa tukada uchit protein folding aur baad mein jatil assembly key liye aavashyak haye. jatil sanyojan key antim charan mein, beeta5आee upa-ikai kaa aine-terminal tukada vighatit hotaa haye, jisase 20aiss parisar kaa paripakva beeta5आee upa-ikai banata haye. besal assembly key dauran, aur ack paripakva upa-ikai utpann karane key liye proteolytic prasanskaran kii aavashyakta hoti haye. upa-ikai beeta5आee kewal pratirakshaatmak protein mein prastut hotaa haye aur 20aiss protesome parisar mein upa-ikai beeta5 (protesome beeta 5 upa-ikai) dwara pratisthaapit kiya jaataa haye.
naidaanik mahatva.
proteojom aur isaki upa-ikaaiyaan cumm seey cumm dou karanon seey naidaanik mahatva kii hainh (1) ack samjhauta kii gaee jatil assembly yaa ack nishkriya proteojom vishisht rogon key antarnihit pathophysiology key saath judaa hoe sakata haye, aur (2) unakaa upayog chikitsiya hastakshepon key liye dawa lakshya key roop mein kiya jaa sakata haye. haal hii mein, nae naidaanik markers aur ranneetiyon key vikas key liye proteojom parr vichaar karane key liye adhik prayaas kiya gayaa haye. proteojom key pathophysiology kii behtar aur vyaapak samajh seey bhavishya mein naidaanik anuprayog honey chaahiye.
proteojoms ubquitin-proteojoms system (upp) aur sambandhit cellular protein gunavatta niyantran (pee. kyu. see.) key liye ack mahatvapurna ghatak banaate hain. protein sarvavyaapi aur baad mein proteolysis aur proteojom dwara ksharan koshika chakr, koshika vriddhi aur vibhedan, jeane pratilekhan, sanket pargaman aur apoptosis key viniyaman mein mahatvapurna tantra hain. baad mein, ack samjhauta kiye gaye proteojom jatil sanyojan aur kaary seey proteolytic gatividhiyon mein kamee aatee haye aur kshatigrast yaa galat tareeke seey modane vaali protein prajaatiyon kaa sanchay hotaa haye. iss tarah key protein kaa sanchay neurodegenerative rogon, hriday rogon, soojan pratikriyaon aur autoimmune rogon mein rogjanan aur phenotypic visheshtaon mein yogdan curr sakata haye, aur pranaaleegat dna kshati pratikriyaon seey ghaatak hoe sakata haye.
kaee prayogaatmak aur naidaanik adhyayanon ney sanket diyaa haye kii upp key vichalan aur viniyaman kaee neurodegenerative aur myodegenerative vikaaron key rogjanan mein yogdan karate hain, jinamen alzheimer roga, parkinsons roga aur pick roga, amyotrophic paarshv sclerosis (ells), huntington roga, cretzfelt-jacob roga, aur motor neuron roga, polyglutamine (polycue) roga, muscular dystrophy aur dementia seey judey neurodegenerative rogon key kaee durlabh roop shamil hain. ubquitin-proteojom pranali (upp) key hisse key roop mein, proteojom cardiac protein homeostasis koo banaae rakhta haye aur iss prakaar cardiac ischemic chot, ventricular hypertrophy aur hriday viphalata mein mahatvapurna bhoomika nibhaata haye. isake alawa, saboot jamaa hoe rahey hain kii upp ghaatak parivartan mein ack aavashyak bhoomika nibhaate hain. ups proteolysis cancer koshikaon kii pratikriyaon mein uttejak sanketon mein ack pramukh bhoomika nibhaata haye joe cancer key vikas key liye mahatvapurna hain. tadnusar, pratilekhan karakon key ksharan dwara jeane abhivyakti, jaise kii pee53, see-juna, see-phos, nf-kebi, see-mike, hif-1α, maitae2, state3, sterol-viniyamit tatva-badhyakari protein aur androgen receptors sabhi upp dwara niyantrit hotey hain aur iss prakaar vibhinn durbhavnaon key vikas mein shamil hotey hain. isake alawa, upp tumour suppressor jeane utpaadon key ksharan koo niyantrit karate hain jaise kii colorectal cancer, retinoblastoma (aar. bee.) mein adenomatous polyposis kolai (aeye. pee. see.). aur wonn hippel-lindaau tumour suppressor (vee. aich. ell.), saath hii kaee proto-oncogenes (aar. aeye. aff., maaee. see., maaee. bee., aar. ell., aiss. aar. see., mose, aeye. bee. ell.). oopar kii or soojan pratikriyaon key viniyaman mein bhee shamil haye. iss gatividhi koo aamtaur parr aine. aff.-κb key sakriyan mein proteojoms kii bhoomika key liye jimmedaar thaharaaya jaataa haye joe aage pro inflammatory cytokine jaise tii. aine. aff.-α, ill-β, ill-8, asanjan anuon (aaee. see. aeye. aim.-1, vee. see. aim.-1, pee.-selectin) aur prostaglandin aur nitric oxide (naheen) kii abhivyakti koo niyantrit kartaa haye. isake alawa, upp bhee leukocyte prasaar key niyamakon key roop mein soojan pratikriyaon mein ack bhoomika nibhaate hain, mukhya roop seey cycline key proteolysis aur cdk avrodhakon key ksharan key madhyam seey. anta mein, slee, sjogren syndrome aur rheumatoid arthuritis (aar. aeye.) vaale autoimmune roga key rogi mukhya roop seey parisanchari protesome pradarshit karate hain jinhen naidaanik biomarker key roop mein lagu kiya jaa sakata haye.
pramukh histocompatibility complexes (aim. aich. see.) varg-aaee key liye pratijan prasanskaran key dauran, protesome pramukh ksharan machinary haye joe pratijan koo cumm karati haye aur parinami peptides koo cytotoxic tii lymphocytes mein prastut karati haye. immunoprotesome koo utpann varg-aaee ligand kii gunavatta aur matra mein sudhaar karane mein mahatvapurna bhoomika nibhaane vaalaa maanaa gayaa haye.
pee. aiss. aim. bee. 8 protein kii autoimmune rogon aur soojan pratikriyaon mein mahatvapurna naidaanik bhoomika haye. udaaharan key liye, immunoprotesome subunit mein ack homozygus missense utparivartan (jee197vee) vaale rogiyon koo, β prakaar 8 (pee. aiss. aim. bee. 8) koo autoinflammatory pratikriyaon seey peedit honah padaa jisamen lipodystrophy key saath baar-baar bukhaar aur nodular erythema shamil they. iss utparivartan ney pratirakshaatmak protesome key sanyojan madhyavarti koo badhaa diyaa, jisake parinamswarup protesome kaary mein kamee aaee aur rogi key ootakon mein sarvavyaapi protein kaa sanchay huaa. rogi kii twacha aur bee koshikaon mein, il-6 bhee atyadhik vyakt kiya gayaa thaa, aur pee. aiss. aim. bee. 8 kii abhivyakti cumm hoe gaee thee. isake alawa, pee. aiss. aim. bee. 8 key downregulation ney vitro mein murine aur maanav edipocytes key antar koo bhee roque diyaa, jabaki choohe kii twacha mein pee. aiss. aim. bee. 8 key khilaf sirna kaa ack injection ediposite ootak kii matra koo cumm curr sakata haye. iss prakaar, pee. aiss. aim. bee. 8 naa kewal soojan key liye ack aavashyak ghatak aur niyaamak hoe sakata haye, balki edipocytes key vibhedan mein bhee hoe sakata haye, jisase yeh sanket milata haye kii immunoprotesome mein vibhinn prakaar kii koshikaon key homeostasis koo banaae rakhane key liye pleotropic kaary hoe sakate hain. baad mein, autoimmune rogon key alawa pee. aiss. aim. bee. 8 protein koo bhee lipodystrophy syndrome key nidan mein jodaa gayaa haye. glycosylation vikaar kabhi-kabhi shamil hotey hain. kuchh aanuvanshik roop seey nirdhaarit roop haal hii mein pee. aiss. aim. bee. 8 key madhyam seey ack proteojom visangati seey judey autoinflammatory syndrome key kaaran paae gaye hain. unake parinamswarup ack lipodystrophy syndrome hotaa haye joe bukhaar, dermatosis aur paniculitis key saath hotaa haye, aur nakajo-nishimura syndrome, ack alag vanshaanugat soojan aur barbad karane vaali bimari joe japan seey utpann hoti haye. nakajo-nishimura syndrome vaale rogiyon mein, aavadhik uchch bukhaar aur nodular erythema jaise visfot hotey hain, aur dheere-dheere oopari shareer mein, mukhya roop seey chehare aur oopari angon mein, chehare kii vishisht patali upasthiti aur jodd anubandh key saath lambi ungaliyon koo dikhaane key liye, lipomuscular atrophy kii pragati hoti haye.

