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Alpha-Mannosidose | SphinCS.de

Der Koala

Alpha-Mannosidose

Alpha-Mannosidose, eine Glykoproteinose, wird durch einen genetischen Defekt des lysosomalen Enzyms alpha-Mannosidase verursacht, das für die Hydrolyse der Kohlehydrat-Seitenketten von Glykoproteinen verantwortlich ist. Die verminderte Aktivität dieses Enzyms führt zur Akkumulation von Oligosacchariden in verschiedenen Zellen und Organen. Alpha-Mannosidose ist eine langsam fortschreitende Erkrankung, die eine breite klinische Variabilität aufweist. Symptome sind eine Hörstörung, Skelett-Anomalien, mentale Retardierung, manchmal auch psychotische Zustände. Weiterhin werden eine Muskelschwäche und eine Ataxie beobachtet, In der ersten Lebensdekade treten rezidivierende Infekte auf, die auf einem Immundefekt beruhen. 

Alpha-Mannosidose | SphinCS - Clinical Science for LSD

Ätiologie: Defizienz der alpha-Mannosidase
Gen: MAN2B1-Gen
Vererbungsmodus: Autosomal-rezessiv
Phänotypen:

Leitsymptome:

Infantile Form
(Manifestationsalter im Säuglingsalter)

Mildere Form
(Manifestationsalter im Kindes-/Jugendalter)

Diagnostik:

Therapie:
Velmanase alfa (Lamzede®)


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