disease 974 words KG: ent-dise-3b1c1078 2026-03-29
kind:diseasesection:diseasestopic:x-ald
Contents

X-Linked Adrenoleukodystrophy (X-ALD) Genetic Variants

Disease Info
PrognosisWithout treatment, most die within 3-5 years of symptom onset
Missense mutations~60% of cases — affect protein folding, trafficking, or function
Nonsense mutations~15% — premature stop codons, complete loss of function
Frameshift mutations~15% — insertions/deletions causing truncations
Splice site mutations~10% — abnormal mRNA processing
Large deletionsRare — complete or partial gene loss
ABCD2Partial functional redundancy with ABCD1, may compensate when ABCD1 is deficient
ABCD3Also can transport VLCFA-CoA, may modify severity
PEX genesPeroxisome biogenesis genes that affect overall peroxisomal function
APOEMay influence cerebral ALD progression
X-linked recessivePrimarily affects males
Female carriersMay develop mild AMN-like symptoms due to skewed X-inactivation (~20-50%)
DatabasesOMIMOrphanetClinicalTrialsPubMed

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