This site uses cookies.
Some of these cookies are essential to the operation of the site,
while others help to improve your experience by providing insights into how the site is being used.
For more information, please see the ProZ.com privacy policy.
Nezávislý překladatel nebo tlumočník, Ověřený uživatel serveru
Data security
This person has a SecurePRO™ card. Because this person is not a ProZ.com Plus subscriber, to view his or her SecurePRO™ card you must be a ProZ.com Business member or Plus subscriber.
angličtina -> nizozemština: Ambroxol and its use in Gaucher disease General field: Věda Detailed field: Biologie (-tech, -chem, mikro-)
Zdrojový text - angličtina Ambroxol is an over-the-counter drug that is used as an expellant of excessive mucus in the respiratory tracts, and as an agent against inflammation and pain in the throat due to coughing. Some years ago, ambroxol was suggested to act as an allosteric pharmacological chaperone for lysosomal glucocerebrosidase 1 (GBA1), the enzyme that is dysfunctional in patients with Gaucher disease (GD). A dysfunction in GBA1 activity results in lysosomal storage of glycosylceramide (GlcCer) and its deacylated form glucosylsphingosine (GlcSph). This lysosomal stress and lipid storage also results in the marked increase of protein biomarkers, such as chitotriosidase (CHIT1) and CC chemokine ligand 18 (CCL18), among others. The severity of the phenotype in GD patients depends on the residual activity of GBA1, and can include hepatosplenomegaly, anemia, thrombocytopenia, bone pain, arthritis and even neurological defects, the latter of which is exclusive to neuropathic Type 2/3 GD. Besides ambroxol’s role as a chaperone, ambroxol was also shown to regulate transcription by activating Transcription factor EB (TFEB), which promotes lysosomal biogenesis. The molecular structure of ambroxol additionally allows it to penetrate the blood-brain barrier (BBB). Taken together, ambroxol could be an attractive new treatment strategy against Type 2/3 GD, which is currently only treatable palliatively.
Překlad - nizozemština Ambroxol is een vrij verkrijgbaar medicijn dat wordt gebruikt tegen overmatig slijm in de luchtwegen, en als middel tegen ontsteking en pijn tijdens het hoesten. Enkele jaren geleden werd gespeculeerd dat ambroxol zou kunnen werken als een allosterische farmacologische chaperonne op lysosomale glucocerebrosidase 1 (GBA1), het enzym dat disfunctioneel is bij patiënten met de ziekte van Gaucher (GD). Een disfunctie in de GBA1-activiteit zorgt voor lysosomale opslag van glycosylceramide (GlcCer) en de gedeacyleerde vorm glucosylsphingosine (GlcSph). Deze lysosomale stress en vetopslag zorgt voor een opmerkelijke toename van eiwitbiomarkers, zoals chitotriosidase (CHIT1) en CC chemokine ligand 18 (CCL18), onder anderen. De ernst van het fenotype bij GD-patiënten hangt af van de resterende activiteit van GBA1. Bij ernstige defecten kan er hepatosplenomegalie, anemie, trombocytopenie, botpijn, artritis en zelfs neurodegeneratie optreden, alhoewel het laatste alleen voorkomt bij neuropathische Type 2/3 GD. Naast de rol van ambroxol als chaperonne werd ook aangetoond dat ambroxol de transcriptie reguleert door Transcription factor EB (TFEB) te activeren, wat de lysosomale biogenese aan zet. De moleculaire structuur van ambroxol maakt het bovendien mogelijk om de bloed-hersenbarrière (BBB) te penetreren. Sammenvattend zou ambroxol een aantrekkelijke nieuwe behandelingsstrategie kunnen zijn tegen Type 2/3 GD; een ziekte die momenteel alleen palliatief behandeld kan worden.
More
Less
Praxe
Počet let praxe: 2. Registrován na ProZ.com: Feb 2023.
Stay up to date on what is happening in the language industry
Help or teach others with what I have learned over the years
Meet new end/direct clients
Build or grow a translation team
Životopis
As an experienced subtitler, successful entrepreneur, and biochemical scientist PhD, my professional experience is both varied and extensive. With my company Glotword, we created a platform with over 400 talented freelancers capable of creating subtitles in 20+ languages. Our team uses state-of-the-art technology to ensure that our subtitles are accurate, timely, and optimized for readability. Whether you're looking to expand your audience, tailor the language style, or simply make your content more accessible, Glotword has the expertise and experience to help you achieve your goals. Contact us today to learn more about our reliable and professional subtitling services.
Specializations:
- Subtitling for movies, series in 20+ languages
- Medical / Biochemical translations for scientific papers, promotions, brochures, and more
- Styled English captioning for Youtube, Tiktok, Instagram 💪