Hla mus rau cov ntsiab lus

Peb qhov kev tshawb fawb cuam tshuam

Isla, uas nyob nrog cov kab mob dystrophic epidermolysis bullosa (RDEB), ua si nrog nws tus dev.
Isla, uas nyob nrog cov kab mob dystrophic epidermolysis bullosa (RDEB).

"Dab tsi kuv cia siab rau EB kev tshawb fawb yog ua kom qhov ua tsis tau ib zaug, ua tau. Kuv xav tau lub neej yav tom ntej rau Isla; Kuv xav tau kev kho mob tshwm sim hauv nws lub neej. "

Andy thiab Isla, DEBRA Cov Tswv Cuab

Peb cov ntaub ntawv tshawb fawb cuam tshuam

Ntawm no ntawm DEBRA UK, Andy & Isla cov hom phiaj kuj yog peb lub hom phiaj. Los ntawm rub tawm koj tus kheej daim ntawv luam ntawm DEBRA UK Kev Tshawb Fawb Txog Kev Tshawb Fawb Txog Kev Tshawb Fawb 2021, koj tuaj yeem paub txog qhov cuam tshuam ntawm epidermolysis ntawm bullosa (EB) ntawm lub neej ntawm ntau txhiab tus menyuam yaus UK, txiv neej thiab poj niam raws li EB cov kws tshawb fawb siv zog nrhiav kev kho.

Nrhiav tau:

  • Cov peev txheej ntawm cov neeg cuam tshuam los ntawm cov kab mob ntawm daim tawv nqaij no debilitating;
  • Peb qhov kev pom zoo rau yav tom ntej tsis pub dawb los ntawm EB;
  • Cov kws kho mob tshwj xeeb thiab cov kev pabcuam muaj rau cov neeg mob EB thiab tsev neeg;
  • Kev cog lus rau kev tshawb fawb zoo sawv cev ntawm EB cov neeg mob thiab tsev neeg;
  • Thiab ntau yam ntxiv. 

Download peb cov ntaub ntawv cuam tshuam 2021

DOWNLOAD our EBS Impact Report

 

Kev lees paub txog nyiaj txiag los ntawm DEBRA UK:

Thaum nthuav tawm lossis tshaj tawm cov txiaj ntsig, cov nyiaj tau los ntawm DEBRA UK yuav tsum tau lees paub siv peb lub logo thiab cov lus: 

' Nyiaj txiag rau - tus lej nyiaj tau txais los ntawm DEBRA UK.'

Tus Thawj Saib Xyuas Kev Tshawb Fawb yuav tsum xa pdf ntawm tag nrho cov ntaub ntawv luam tawm, xa cov ntawv sau thiab cov ntaub ntawv hais txog kev sib tham txog Txoj Haujlwm rau DEBRA UK thoob plaws lub sijhawm nyiaj pab thiab rau tsib xyoos tom qab qhov nyiaj pab tas. Cov ntawv tshaj tawm yuav muaj nyob hauv qab no:

Cov ntawv tshaj tawm los ntawm DEBRA UK nyiaj txiag

DEBRA UK Project Kev tshaj tawm kev tshawb fawb Nqe lus piav qhia

KEB and skin cancer (2024)

 Kev koom tes ntawm Kindlin-1 hauv cutaneous squamous cell carcinoma  

PEBLES RDEB Symptoms Study (2022)

 Mob nyob rau hauv recessive dystrophic epidermolysis bullosa (RDEB): kev tshawb pom ntawm Prospective Epidermolysis Bullosa Longitudinal Evaluation Study (PEBLES) Mob ntau, tab sis nyuaj rau tswj, hauv RDEB: kawm

Permanent gene therapy for RDEB

 Cov tshuaj gene kho kho siv lipid nanoparticles: 'gene creams' rau kab mob ntawm daim tawv nqaij? 'Gene cream' rau cov kab mob caj ces

Permanent gene therapy for RDEB

 Lipid Nanoparticles Ua tau zoo xa cov Base Editor ABE8e rau COL7A1 Kho hauv Dystrophic Epidermolysis Bullosa Fibroblasts Hauv Vitro  

Daim tawv nqaij microbiome ntawm txhua hom EB (2023)

Kev kho mob hauv txhua hom EB (2023)

 Genotype-phenotype correlation hauv Junctional Epidermolysis Bullosa: cov cim qhia rau qhov hnyav Cov kev hloov pauv JEB tshwj xeeb piav qhia dav dav hauv kev kho mob, kev tshawb fawb qhia
Nkag siab txog kab mob ntawm txoj hlab ntsws hauv JEB (2023) Ntawm LAMA3 thiab LAMB3: Ib txoj kev kho noob tshiab rau epidermolysis bullosa (Cov lus qhia txog kev ua haujlwm luam tawm xyoo 2024)  
Nkag siab txog kab mob ntawm txoj hlab ntsws hauv JEB (2023) Lentiviral qhia ntawm wildtype LAM 3A restores cell adhesion nyob rau hauv cov hlab cua basal hlwb los ntawm cov me nyuam nrog epidermolysis bullosa Kev kho mob ntawm tes thiab noob caj noob ces qhia kev cog lus rau EB cov menyuam yaus
Scarring in RDEB (2023) Gamma-Secretase Inhibitors Downregulate Profibrotic NOTCH Signaling Pathway nyob rau hauv Recessive Dystrophic Epidermolysis Bullosa Thaiv NOTCH teeb liab txoj hauv kev tuaj yeem yooj yim RDEB caws pliav: Kawm
DEBRA UK Project Kev tshaj tawm kev tshawb fawb Nqe lus piav qhia
PEBLES RDEB Symptoms Study (2022) khaus nyob rau hauv recessive dystrophic epidermolysis bullosa: kev tshawb pom ntawm PEBLES, kev tshawb fawb txog kev sau npe yav tom ntej Khaus khaus muaj nyob rau hauv RDEB – muaj kev kho mob tsis pab ntau
PEBLES RDEB Symptoms Study (2022) Tus nqi ntawm UK kev saib xyuas hauv zej zog rau cov tib neeg uas muaj cov kab mob dystrophic epidermolysis bullosa: Kev Tshawb Fawb Txog Kev Tshawb Fawb Txog Epidermolysis Bullosa Longitudinal Evaluation Study  
Kev kho mob hauv txhua hom EB (2023) IgA nephropathy nyob rau hauv cov neeg laus nrog epidermolysis bullosa  
Kev kho mob hauv txhua hom EB (2023) Puas yog cov qhab nia raug lees paub qhov hnyav rau epidermolysis bullosa cuam tshuam txog lub nra ntawm tus kab mob hauv cov neeg mob epidermolysis bullosa simplex?  
Kev koom tes hauv lub cev tiv thaiv kab mob hauv RDEB (2023) Inflammation-mediated fibroblast activation thiab kev tiv thaiv kab mob hauv collagen VII-daim tawv nqaij Kev mob hauv RDEB tuaj yeem ua rau mob qog noj ntshav ntawm daim tawv nqaij 
Kev kho mob hauv txhua hom EB (2023) 967 Spatiotemporal hloov ntawm cov zej zog microbial ntawm ntau theem ntawm kev kho mob hauv epidermolysis bullosa  
Kev kho mob hauv txhua hom EB (2023) BG05 Artificial txawj ntse twv kwv yees ntawm splice qhov chaw hloov pauv qhov tshwm sim hauv junctional epidermolysis bullosa: cov cim qhia rau qhov hnyav  
Kev kho mob hauv txhua hom EB (2023) BG06 Tshaj keratin: thaum simplex tsis yooj yim li

 

DEBRA UK Project Kev tshaj tawm kev tshawb fawb
Immune system cells thiab RDEB qhov txhab (2022) Lub luag hauj lwm ntawm mTOR Signaling Cascade nyob rau hauv Epidermal Morphogenesis thiab daim tawv nqaij Barrier tsim
Taug kev nrog EBS (2022) P35: Kev hloov pauv ntawm lub zog siv hauv qab ko taw hauv cov neeg mob epidermolysis bullosa simplex thaum taug kev

Taug kev nrog EBS (2022)

Kev kho mob hauv txhua hom EB (2023)

 488 Hauv av cov tshuaj tiv thaiv rog (GRF) hauv cov neeg mob epidermolysis bullosa simplex (EBS) thaum taug kev
Txau ntawm RDEB gene therapy (2022) Kev tshuaj xyuas cov pej xeem-raws li genotype-phenotype correlations hauv recessive dystrophic epidermolysis bullosa (RDEB)
Txau ntawm RDEB gene therapy (2022) Restoring hom VII collagen nyob rau hauv daim tawv nqaij
Kev kho Cannabinoid rau txhua qhov mob EB thiab khaus Txoj kev tshawb no C4EB-Transvamix (10% THC / 5% CBD) los kho qhov mob ntev hauv epidermolysis bullosa: Ib txoj cai rau kev tshawb nrhiav randomized, placebo tswj, thiab ob chav dig muag kev cuam tshuam crossover kev kawm
Kev kho mob hauv txhua hom EB (2023) Localized autosomal recessive epidermolysis bullosa simplex tshwm sim los ntawm ib qho tshiab homozygous frameshift mutation hauv DST (BPAG1)
Kev kho mob hauv txhua hom EB (2023) P17: Kev hloov pauv kev saib xyuas hauv epidermolysis bullosa: tus qauv rau kev kho mob dav dav
Kev kho mob hauv txhua hom EB (2023) BG11: Scratching lub ocular nto: rov qab saib xyuas ntawm ophthalmological manifestations nyob rau hauv epidermolysis bullosa
Kev kho mob hauv txhua hom EB (2023) BG12: Cicatricial junctional epidermolysis bullosa: ib tug tsis nco qab subtype
Kev kho mob hauv txhua hom EB (2023) LB978 Cov txheeb ze ntau ntawm bacillales yog txuam nrog epidermolysis bullosa (EB) ntawm ntau theem ntawm qhov txhab kho
Kev kho mob hauv txhua hom EB (2023) P33: Influencers nyob rau hauv epidermolysis bullosa: cutaneous microbiome

 

DEBRA UK Project Kev tshaj tawm kev tshawb fawb Nqe lus piav qhia
Kho DEB khaus nrog qia hlwb (2022) Prevalence, pathophysiology thiab kev tswj cov khaus hauv epidermolysis bullosa  
Kho DEB khaus nrog qia hlwb (2022) Transcriptomic profiling ntawm recessive dystrophic epidermolysis bullosa raug mob ntawm daim tawv nqaij highlights tshuaj repurposing lub sij hawm los txhim kho qhov txhab. Methotrexate tuaj yeem pab kho daim tawv nqaij hauv RDEB, kev tshawb fawb qhia
Immune system cells thiab RDEB qhov txhab (2022) Txoj Cai ntawm Txoj Kev Kho Hniav Thaum Ntxov  
Immune system cells thiab RDEB qhov txhab (2022) Mitochondrial metabolism ua haujlwm kho cov txheej txheem tshwj xeeb hauv macrophages thaum kho qhov txhab.  
Kev kho qhov ncauj / caj pas (2022) Tsawg Acyl Gellan ua ib qho Excipient los txhim kho cov tshuaj tsuag thiab mucoadhesion ntawm Iota Carrageenan hauv qhov ntswg qhov ntswg los tiv thaiv kev kis mob nrog SARS-CoV-2  
Txau ntawm RDEB gene therapy (2022) Lub peev xwm ntawm kev kho noob rau recessive dystrophic epidermolysis bullosa  

Repurposing anti-scarring tshuaj nyob rau hauv RDEB

Scarring in RDEB (2024)

 Notch-ing txog kev paub txog cov txheej txheem molecular ntawm daim tawv nqaij fibrosis: tsom mus rau ntau qhov sib txawv Notch signaling txoj kev  
Kev kho mob hauv txhua hom EB (2023) Diagnostic kab mob ntawm daim tawv nqaij  
Kev kho mob hauv txhua hom EB (2023) P45: Qhov zoo, qhov tsis zoo thiab qhov tsis zoo: qhov mob ntawm epidermolysis bullosa qhov txhab  
Kev kho mob hauv txhua hom EB (2023) BG08: JEBseq: cov ntaub ntawv tshiab los tshawb txog genotype-phenotype correlation hauv junctional epidermolysis bullosa

 

DEBRA UK Project Kev Tshaj Tawm Txog Kev Tshawb Fawb Nqe lus piav qhia
Immune system cells thiab RDEB qhov txhab (2022) Glutamine Metabolism Tswj Stem Cell Fate Reversibility thiab Kev Kho Lub Sij Hawm Ntev hauv Cov plaub hau Follicle  
RDEB daim tawv nqaij mob cancer (2023) Heterogeneous addiction rau transforming loj hlob yam-beta signaling nyob rau hauv recessive dystrophic epidermolysis bullosa-koom nrog cutaneous squamous cell carcinoma  
Kev kho mob hauv txhua hom EB (2023) Epidermolysis bullosa

 

DEBRA UK Project Kev Tshaj Tawm Txog Kev Tshawb Fawb Nqe lus piav qhia
Immune system cells thiab RDEB qhov txhab (2022) Epidermal mammalian lub hom phiaj ntawm rapamycin complex 2 tswj lipid synthesis thiab filaggrin ua hauv epidermal barrier tsim  
Rigosertib rau RDEB SSC (2022) Kev txheeb xyuas ntawm Rigosertib rau Kev Kho Mob Recessive Dystrophic Epidermolysis Bullosa-Associated Squamous Cell Carcinoma Rigosertib tuaj yeem ua tau zoo, muaj kev nyab xeeb rau squamous cell carcinoma hauv cov neeg mob RDEB, txoj kev tshawb fawb qhia

 

 

Peb yuav nyiam mloog los ntawm koj!

Peb xav hnov ​​​​cov suab ntawm cov tsev neeg nyob nrog EB los pab peb txiav txim siab seb qhov kev tshawb fawb twg los pab nyiaj.

Yog tias koj xav qhia rau peb paub koj cov kev xav txog peb cov kev tshawb fawb los yog zoo siab rau peb hu rau koj kom nug koj cov kev xav txog qhov kev tshawb fawb peb tau txais, thov koom tes.