FrancophonIA/ELRC3.0 huggingface dataset:
English: Potential interactions with other medicinal products
Slovenian: Morebitno medsebojno delovanje z drugimi zdravili
English: Healthy volunteers receiving a single oral dose of 200 mg/kg of ataluren experienced transient, low-grade symptoms of headache, nausea, vomiting, and diarrhoea.
Slovenian: Pri zdravih prostovoljcih, ki so prejeli enkratni peroralni odmerek atalurena 200 mg/kg, so se pojavili prehodni blažji simptomi, med katerimi so bili glavobol, slabost, bruhanje in driska.
English: The safety, pharmacokinetics and exploratory effectiveness of Translarna were assessed in an openlabel study in children between 2 and 5 years of age with nmDMD.
Slovenian: Pediatrična populacija Varnost, farmakokinetiko in raziskovalno učinkovitost zdravila Translarna so ocenili v odprti študiji z otroki, starimi od 2 do 5 let, z nmDMD.
English: Therefore, caution should be exercised when ataluren is co-administered with medicinal products that are substrates of OAT1 or OATP1B3 because of the risk of increased concentration of these medicinal products (eg oseltamivir, aciclovir, captopril, furosemide, bumetanide, valsartan, pravastatin, rosuvastatin, atorvastatin, pitavastatin).
Slovenian: Zato je pri sočasni uporabi atalurena z zdravili, ki so substrati OAT1 ali OATP1B3, potrebna previdnost, saj obstaja tveganje za zvišane koncentracije teh zdravil (to so npr. oseltamivir, aciklovir, kaptopril, furosemid, bumetanid, valsartan, pravastatin, rosuvastatin, atorvastatin in pitavastatin).
English: For example, you might take Translarna at 7:00 AM in the morning with breakfast, at 1:00 PM in the afternoon with lunch, and again at around 7:00 PM in the evening with dinner.
Slovenian: Na primer: zdravilo Translarna lahko vzamete ob 7. uri zjutraj med zajtrkom, ob 13. uri popoldan med kosilom in nato še okoli 19. ure zvečer med večerjo.
English: An open-label study was performed including patients aged 2-5 years (n=14) to evaluate the PK and safety of ataluren.
Slovenian: Izvedli so odprto študijo, v katero so bili vključeni bolniki, stari od 2 do 5 let (n = 14) za ovrednotenje farmakokinetike in varnosti atalurena.
English: Your doctor will test the levels of lipids (fats such as cholesterol and triglycerides) in your blood and your kidney function every 6 to 12 months.
Slovenian: Zdravnik bo vsakih 6 do 12 mesecev preveril ravni lipidov (maščob, kot so holesterol in trigliceridi) v vaši krvi ter delovanje vaših ledvic.
English: In the ITT population, the hazard ratio for ataluren versus placebo was 0.75 (p=0.160), representing a 25% reduction in the risk of 10% 6MWD worsening.
Slovenian: V populaciji ITT je bilo razmerje tveganja za ataluren v primerjavi s placebom 0,75 (p = 0,160), kar predstavlja 25-% zmanjšanje tveganja za 10-% poslabšanje v 6MWD.
English: Changes in lipid profile
Slovenian: Spremembe ravni lipidov
English: The most common adverse reactions in the 2 placebo-controlled studies were vomiting, diarrhoea, nausea, headache, upper abdominal pain, and flatulence, all occurring in ≥5% of all ataluren-treated patients.
Slovenian: Najpogostejši neželeni učinki v 2 s placebom kontroliranih študijah so bili bruhanje, driska, navzea, glavobol, bolečine v zgornjem delu trebuha in flatulenca, ki so se vsi pojavili pri ≥ 5 % bolnikov, zdravljenih z atalurenom.
English: If you forget to take Translarna
Slovenian: Če ste pozabili vzeti zdravilo Translarna
English: Therefore, if treatment with intravenous aminoglycosides is necessary the treatment with Translarna should be stopped and can be resumed 2 days after administration of the aminoglycoside has ended.
Slovenian: Če je torej potrebno zdravljenje z aminoglikozidi za intravensko uporabo, je treba zdravljenje z zdravilom Translarna prekiniti, ponovno pa se lahko uvede dva dneva po koncu uporabe aminoglikozidov.
English: - If you are receiving treatment with certain antibiotics, such as gentamicin, tobramycin, or streptomycin by injection into a vein.
Slovenian: - če prejemate zdravljenje z določenimi antibiotiki, kot so gentamicin, tobramicin ali streptomicin, ki se dajejo z injiciranjem v veno.
English: Background incidence of this tumour type in rats as well as humans is very low and the mechanism causing these tumours in the rat studies (including its relation to ataluren treatment) is unknown.
Slovenian: Izkušnje kažejo, da je incidenca te vrste tumorja pri podganah in pri ljudeh zelo majhna, mehanizem, ki je v študijah pri podganah povzročil omenjene tumorje, pa ni znan (niti njegova povezava s prejemanjem atalurena).
English: Since the mechanism by which ataluren increases nephrotoxicity of intravenous aminoglycosides is not known, concomitant use of other nephrotoxic medicinal products with ataluren is not recommended.
Slovenian: Ker mehanizem, preko katerega ataluren poveča nefrotoksičnost aminoglikozidov za intravensko uporabo, ni znan, sočasna uporaba drugih nefrotoksičnih zdravil in atalurena ni priporočljiva.
English: Therefore, patients with severe renal impairment or end-stage renal disease should be treated with ataluren only if the anticipated clinical benefit outweighs the potential risk, and should be closely monitored for possible metabolite toxicity and decrease in efficacy.
Slovenian: Zato je treba bolnike s hudo okvaro ledvic ali končno odpovedjo ledvic zdraviti z atalurenom le, če pričakovane klinične koristi pretehtajo tveganje, in treba jih je skrbno spremljati glede potencialne toksičnosti presnovka in zmanjšanja učinkovitosti.
English: There are no adequate data from the use of ataluren in pregnant women.
Slovenian: O uporabi atalurena pri nosečnicah ni zadostnih podatkov.
English: The prepared dose should be mixed well before administration.
Slovenian: Pripravljen odmerek je treba pred dajanjem dobro premešati.
English: Concomitant use of intravenous aminoglycosides (see sections 4.4 and 4.5).
Slovenian: Sočasna uporaba aminoglikozidov za intravensko uporabo (glejte poglavji 4.4 in 4.5).
English: Sachets should only be opened at the time of dose preparation.
Slovenian: Vrečice se lahko odprejo šele takrat, ko je čas za pripravo odmerka.
English: Based on a pharmacokinetic assessment conducted in groups with either mild, moderate or severe hepatic impairment versus a control group of healthy subjects, no dose adjustment is required for patients with any degree of hepatic impairment.
Slovenian: Na podlagi farmakokinetične ocene, izvedene v skupinah z blagimi, zmernimi ali hudimi okvarami jeter v primerjavi s kontrolno skupino zdravih preiskovancev, prilagajanje odmerka pri bolnikih z nobeno stopnjo okvare jeter ni potrebno.
English: Caution should be exercised when ataluren is co-administered with medicinal products that are inducers of UGT1A9, or substrates of OAT1 or OAT3 (see section 4.5).
Slovenian: Previdnost je potrebna, kadar se ataluren uporablja sočasno z zdravili, ki so induktorji UGT1A9 ali substrati OAT1 ali OAT3 (glejte poglavje 4.5).
English: Coadministration of corticosteroids (deflazacort, prednisone, or prednisolone) with ataluren did not affect the plasma concentrations of ataluren.
Slovenian: Sočasna uporaba atalurena in kortikosteroidov (deflazakorta, prednizona ali prednizolona) ni vplivala na koncentracije atalurena v plazmi.
English: The presence of a nonsense mutation in the dystrophin gene should be determined by genetic testing (see section 4.4).
Slovenian: Prisotnost nesmiselne mutacije gena za distrofin je treba potrditi z genetskimi preiskavami (glejte poglavje 4.4).
English: In a pharmacokinetic study in subjects with varying degrees of renal impairment, following a single dose administration, ataluren plasma exposure changed by -13%, 27%, and 61% for the mild, moderate and severe groups, respectively, and 46% for the end-stage renal disease group compared with the normal renal function group.
Slovenian: V farmakokinetični študiji se je pri preiskovancih z različnimi stopnjami okvare ledvic po uporabi enkratnega odmerka plazemska izpostavljenost atalurenu spremenila za -13 %, 27 % in 61 % pri skupinah z blago, zmerno in hudo okvaro ter za 46 % pri skupini s končno odpovedjo ledvic v primerjavi s skupino z normalnim delovanjem ledvic.
English: However there were no apparent effects of gender on ataluren plasma exposure in other populations.
Slovenian: Vendar pri drugih populacijah niso opazili, da bi spol vplival na izpostavljenost atalurenu v plazmi.
English: In rats and rabbits embryo-foetal toxicity (e.g. increased early resorptions, postimplantation loss, decreased viable foetuses) and signs of delayed development (increased skeletal variations) were found in the presence of maternal toxicity.
Slovenian: Pri podganah in kuncih so opazili embriofetalno toksičnost (npr. večje število zgodnjih resorpcij in poimplantacijskih izgub ter manjše število živih zarodkov) ter znake zaostalosti v razvoju (večje število skeletnih sprememb), kadar je bila prisotna toksičnost pri materi.
English: The first dose should be taken in the morning, the second at midday, and the third in the evening.
Slovenian: Prvi odmerek je treba vzeti zjutraj, drugega zgodaj popoldne in tretjega zvečer.
English: Studies in animals have shown reproductive toxicity only at doses that resulted in maternal toxicity (see section 5.3).
Slovenian: Študije na živalih so pokazale vpliv na sposobnost razmnoževanja samo pri odmerkih, ki so povzročili toksičnost pri materi (glejte poglavje 5.3).
English: The efficacy and safety of Translarna were assessed in 2 randomised, double-blind, placebo-controlled, trials in nmDMD.
Slovenian: Učinkovitost in varnost zdravila Translarna so ocenjevali v 2 randomiziranih, dvojno slepih, s placebom kontroliranih preskušanjih o nmDMD.
English: Almac Pharma Services 22 Seagoe Industrial Estate Craigavon BT63 5QD United Kingdom
Slovenian: 22 Seagoe Industrial Estate Craigavon BT63 5QD Velika Britanija
English: In a statistical based model the estimated mean difference was 31.7 meters (adjusted p=0.0367).
Slovenian: Pri oceni statističnega modela je bila povprečna razlika 31,7 metra (prilagojena vrednost p = 0,0367).
English: Treatment should not be initiated in previously untreated patients with eGFR <30 ml/min (see sections 4.2 and 5.2).
Slovenian: Zdravljenja ni dovoljeno začeti pri predhodno nezdravljenih bolnikih z eGFR < 30 ml/min (glejte poglavji 4.2 in 5.2).
English: The significance of these findings for humans is unknown.
Slovenian: Pomen tega izsledka pri ljudeh ni znan.
English: Co-administration of rifampicin, a strong inducer of metabolic enzymes including UGT1A9, decreased ataluren exposure by 29%.
Slovenian: Sočasna uporaba rifampicina, močnega induktorja presnovnih encimov, vključno z UGT1A9, zmanjša izpostavljenost atalurenu za 29 %.
English: Based on data from subjects ranging in age from 2 years to 57 years, there is no apparent effect of age on ataluren plasma exposure.
Slovenian: Podatki, pridobljeni pri osebah, starih od 2 do 57 let, ne kažejo, da bi starost vplivala na izpostavljenost atalurenu v plazmi.
English: Adverse reactions were generally mild or moderate in severity, and no treatment-related serious adverse events were reported among ataluren-treated patients in these 2 studies.
Slovenian: Na splošno so bili neželeni učinki po resnosti blagi ali zmerni; pri bolnikih, zdravljenih z atalurenom, v teh 2 študijah niso poročali o resnih neželenih učinkih.
English: Available pharmacodynamic/toxicological data in animals have shown excretion of ataluren/metabolites in milk (see section 5.3).
Slovenian: Razpoložljivi farmakodinamični/toksikološki podatki pri živalih kažejo na izločanje atalurena/presnovkov v mleko (glejte poglavje 5.3).
English: The table below provides information on which sachet strength(s) to use in the preparation of the recommended dose by body weight range.
Slovenian: V spodnji razpredelnici so podatki o jakosti(h) vrečic, ki jih je treba uporabiti za pripravo priporočenega odmerka glede na razpon telesne mase.
English: Exposure at the no observed adverse effect level (NOAEL) was similar to (rabbit) or 4 times (rat) the systemic exposure in humans (40 mg/kg/day).
Slovenian: Izpostavljenost pri odmerku, pri katerem ni mogoče opaziti neželenih učinkov (NOAEL), je bila podobna (pri kuncih) sistemski izpostavljenosti človeka (40 mg/kg/dan) ali 4-krat večja (pri podganah).
English: These may affect your kidney function.
Slovenian: To bi lahko vplivalo na delovanje ledvic.
English: Translarna is indicated for the treatment of Duchenne muscular dystrophy resulting from a nonsense mutation in the dystrophin gene, in ambulatory patients aged 2 years and older (see section 5.1).
Slovenian: Zdravilo Translarna je indicirano za zdravljenje Duchennove mišične distrofije, ki je posledica nesmiselne mutacije gena za distrofin, pri mobilnih bolnikih, starih 2 leti ali več (glejte poglavje 5.1).
English: In particular do not take Translarna with the antibiotics gentamicin, tobramycin, or streptomycin given by injection.
Slovenian: Zlasti ne jemljite zdravila Translarna skupaj z antibiotiki gentamicinom, tobramicinom ali streptomicinom, ki se dajejo z injiciranjem.
English: The European Medicines Agency will review new information on this medicine at least every year and this leaflet will be updated as necessary.
Slovenian: Evropska agencija za zdravila bo vsaj enkrat letno ponovno pregledala nove podatke o zdravilu in posodobila povzetek glavnih značilnosti zdravila, če bo potrebno.
English: A higher frequency of malaise (7.1%), pyrexia (42.9%), ear infection (28.6%), and rash (21.4%) were reported in patients aged 2-5 years compared with patients 5 years of age and older.
Slovenian: Pri bolnikih, starih od 2 do 5 let, so opazili večjo pogostnost splošnega slabega počutja (7,1 %), pireksije (42,9 %), okužbe ušesa (28,6 %) in izpuščaja (21,4 %) kot pri bolnikih, starih 5 let in več.
English: Ataluren enables ribosomal readthrough of mRNA containing such a premature stop codon, resulting in production of a full-length protein.
Slovenian: Ataluren omogoči, da ribosomi podaljšajo translacijo mRNA, ki vsebuje tak prezgodnji zaključni kodon, zaradi česar lahko nastane beljakovina s polno dolžino.
English: Adverse reactions reported in >1 patient in the 40 mg/kg/day group at a frequency greater than that of the placebo group are presented by MedDRA System Organ Class, Preferred Term, and frequency.
Slovenian: Neželeni učinki, o katerih so poročali pri > 1 bolnikov v skupini 40 mg/kg/dan in z večjo pogostnostjo kot v skupini s placebom, so predstavljeni po po organskih sistemih po MedDRA, prednostnih izrazih in pogostnosti.
English: 26% worsening
Slovenian: poslabšanje pri 26 % poslabšanje pri 44 %
English: The effect of ataluren on driving, on cycling, or on using machines has not been tested.
Slovenian: Vpliv atalurena na sposobnost vožnje, kolesarjenja ali upravljanja strojev ni bil raziskan.
English: If you stop taking Translarna
Slovenian: Če ste prenehali jemati zdravilo Translarna
English: Steady-state plasma concentrations are dose-proportional for ataluren doses between 10 and 50 mg/kg, and no accumulation is observed after repeated dosing.
Slovenian: Plazemske koncentracije v stanju dinamičnega ravnovesja so sorazmerne z odmerkom pri odmerkih atalurena od 10 do 50 mg/kg, pri ponavljajočem se odmerjanju pa ne prihaja do kopičenja.
English: Duchenne muscular dystrophy is caused by genetic changes that result in an abnormality in a muscle protein called dystrophin which is needed for muscles to work properly.
Slovenian: Duchennovo mišično distrofijo povzročijo genetske spremembe, ki so posledica anomalije mišične beljakovine, imenovane distrofin, ki je potrebna za pravilno delovanje mišic.
English: Patients who do not have a nonsense mutation should not receive ataluren.
Slovenian: Bolniki brez nesmiselne mutacije ne smejo prejemati atalurena.
English: There were no apparent differences in either steady-state relative bioavailability or apparent clearance due to loss of ambulation.
Slovenian: Opaznih razlik v relativni biološki uporabnosti v stanju dinamičnega ravnovesja ali navideznem očistku zaradi izgube mobilnosti ni bilo.
English: An increase in serum lipids, i.e. cholesterol and triglycerides, was observed.
Slovenian: Opazili so povečanje vrednosti serumskih lipidov, tj. holesterola in trigliceridov.
English: General characteristics of ataluren after administration
Slovenian: Splošne značilnosti atalurena po dajanju
English: atorvastatin lipid-lowering benzylpenicillin severe infections bumetanide treatment or prevention of congestive heart failure captopril treatment or prevention of congestive heart failure ciprofloxacin treatment of infections famotidine treatment of active duodenal ulcer, gastroesophageal reflux disease furosemide treatment or prevention of congestive heart failure methotrexate rheumatoid arthritis, psoriasis olmesartan essential hypertension in adults oseltamivir prevention of influenza phenobarbital sleep-inducing, prevention of seizures pitavastatin lipid-lowering pravastatin lipid-lowering rifampicin treatment for tuberculosis rosuvastatin lipid-lowering sitagliptin type 2 diabetes valsartan treatment or prevention of congestive heart failure Some of these medicines were not tested together with Translarna and your doctor may decide to monitor you closely.
Slovenian: atorvastatin zniževanje ravni lipidov benzilpenicilin hude okužbe bumetanid zdravljenje ali preprečevanje kongestivnega srčnega popuščanja kaptopril zdravljenje ali preprečevanje kongestivnega srčnega popuščanja ciprofloksacin zdravljenje okužb famotidin zdravljenje aktivne razjede dvanajstnika ali gastroezofagealne refluksne bolezni furosemid zdravljenje ali preprečevanje kongestivnega srčnega popuščanja metotreksat revmatoidni artritis, luskavico olmesartan esencialno hipertenzijo pri odraslih oseltamivir preprečevanje gripe fenobarbital pomoč pri spanju, preprečevanje epileptičnih napadov pitavastatin zniževanje ravni lipidov pravastatin zniževanje ravni lipidov rifampicin zdravljenje tuberkuloze rosuvastatin zniževanje ravni lipidov sitagliptin sladkorno bolezen tipa 2
English: Paediatric patients with body weight ≥12 kg are treated as per the dosing recommendations by body weight range (see above dosing table).
Slovenian: Pediatrična populacija Pediatrični bolniki s telesno maso ≥ 12 kg se zdravijo po priporočilih za odmerjanje in glede na razpon telesne mase (glejte zgornjo preglednico za odmerjanje).
English: Also rat and dog studies showed changes in plasma lipid (cholesterol and triglycerides) suggestive of changes in fat metabolism.
Slovenian: Poleg tega so študije pri podganah in psih pokazale spremembe plazemskih lipidov (holesterola in trigliceridov), ki so znak sprememb v presnovi maščob.
English: A 1-year study in 10-12 weeks old dogs demonstrated findings in the adrenal gland (focal inflammation and degeneration in the glucocorticoid-producing regions of the cortex) and a mild compromise of cortisol production after exogenous stimulation with adrenocorticotropic hormone.
Slovenian: 1-letna študija pri psih, starih od 10-12 tednov, je pokazala spremembe v nadledvični žlezi (žariščno vnetje in degeneracija predelov skorje, kjer nastajajo glukokortikoidi) in manjši negativen vpliv na proizvodnjo kortizola po eksogeni stimulaciji z adrenokortikotropnim hormonom.
English: The prepared dose should be discarded if not consumed within 24 hours of preparation if kept refrigerated (2 - 8 °C), or within 3 hours at room temperature (15 - 30 °C).
Slovenian: Pripravljen odmerek je treba zavreči, v kolikor se ni uporabil v 24 urah po pripravi, če je bil shranjen v hladilniku (2-8 °C), oziroma v 3 urah, če je bil shranjen pri sobni temperaturi (15-30 °C).
English: 6MWD Results in Patients with a Baseline 6MWD < 350 meters.
Slovenian: Rezultati merjenja 6MWD pri bolnikih z izhodiščno 6MWD < 350 metrov
English: Nonclinical in vitro studies suggest that continuous exposure to ataluren may be important for maximizing activity and that effects of the active substance on ribosomal read-through of premature stop codons reverse shortly after withdrawal of ataluren.
Slovenian: Predklinične študije in vitro kažejo, da bi lahko bila neprekinjena izpostavljenost atalurenu pomembna za to, da se doseže čim večja aktivnost, in da učinek učinkovine na ribosome, da podaljšajo translacijo preko prezgodnjega zaključnega kodona, izgine kmalu po prekinitvi zdravljenja z atalurenom.
English: There was no difference between ataluren 80 mg/kg/day and placebo.
Slovenian: Med atalurenom 80 mg/kg/dan in placebom ni bilo razlike.
English: In a clinical study, the extent of exposure for ciprofloxacin was 32% higher in the presence of ataluren.
Slovenian: V klinični študiji je bil obseg izpostavljenosti za ciprofloksacin v prisotnosti alaturena 32 % večji.
English: At a single tested, relatively low, maternal dose of 30 mg/kg, the concentration of foetal radioactivity was ≤ 27% of the maternal concentration.
Slovenian: Pri preskušanju enkratnega relativno majhnega odmerka 30 mg/kg, ki so ga dali materi, je koncentracija radioaktivnosti pri plodu znašala ≤ 27 % koncentracije pri materi.
English: No dosing adjustment is needed for patients who are becoming nonambulatory.
Slovenian: Prilagajanje odmerka pri bolnikih, ki postajajo imobilni, ni potrebno.
English: In a separate clinical study, the extent of exposure for adefovir was 60% higher in the presence of ataluren.
Slovenian: V ločeni klinični študiji je bil obseg izpostavljenosti za adefovir v prisotnosti atalurena 60 % večji.
English: In the clinical program investigating the efficacy and safety of monotherapy ataluren in patients with nonsense mutation cystic fibrosis, no statistically significant effect was observed in the primary and key secondary clinical outcome measures (ppFEV1 and pulmonary exacerbation rate) in adults and children aged 6 years and older.
Slovenian: V kliničnem programu za preučevanje učinkovitosti in varnosti monoterapije z atalurenom pri bolnikih z nesmiselno mutacijo cistične fibroze, pri odraslih in otrocih, starih 6 let in več niso opazili statistično pomembnega učinka pri primarnih in ključnih sekundarnih merilih kliničnega izida (ppFEV1 in stopnja pljučnega poslabšanja).
English: Effect of ataluren on pharmacokinetics of other medicinal products Based on in vitro studies, ataluren has the potential to inhibit UGT1A9, organic anion transporter 1 (OAT1), organic anion transporter 3 (OAT3) and organic anion transporting polypeptide 1B3 (OATP1B3).
Slovenian: Vpliv atalurena na farmakokinetiko drugih zdravil Študije in vitro so pokazale, da ima ataluren potencial za zaviranje UGT1A9, prenašalca organskih anionov 1 (OAT1), prenašalca organskih anionov 3 (OAT3) in polipeptidnega prenašalca organskih anionov 1B3 (OATP1B3).
English: Translarna is used to treat Duchenne muscular dystrophy resulting from a specific genetic defect that affects normal muscle function.
Slovenian: Zdravilo Translarna se uporablja za zdravljenje Duchennove mišične distrofije, ki je posledica posebne genetske okvare, ki vpliva na normalno delovanje mišic.
English: If this is unavoidable (e.g. vancomycin to treat MRSA) careful monitoring of renal function is advised (see section 4.5).
Slovenian: Če so ta zdravila nujna (npr. vankomicin za zdravljenje MRSA), je priporočljivo skrbno spremljati delovanje ledvic (glejte poglavje
English: Medicine
Slovenian: ZDRAVILA
English: The co-administration of these medicinal products with ataluren should be avoided (see section 4.3).
Slovenian: Sočasni uporabi teh zdravil in atalurena se je treba izogibati (glejte poglavje 4.3).
English: Ataluren plasma concentrations at steady state increase proportionally with increasing dose.
Slovenian: Plazemske koncentracije atalurena v stanju dinamičnega ravnovesja naraščajo sorazmerno z velikostjo odmerka.
English: Each prepared dose is best administered immediately after preparation.
Slovenian: 4 leta Posamezen pripravljen odmerek je najbolje dati takoj po pripravi.
English: Translarna enables the production of working dystrophin and helps muscles work properly.
Slovenian: Zdravilo Translarna omogoči nastajanje pravilno delujočega distrofina in pomaga mišicam pri pravilnem delovanju.