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항목
CGTCurated CoreFDAApproved
atidarsagene autotemcel (Lenmeldy, OTL-200, Libmeldy, arsa-cel)
Orchard Therapeutics (Kyowa Kirin)·ARSA (arylsulfatase A) gene
3 trials·t½ Durable engraftment; ARSA activity restored within months of infusion
Overview
Program
Lenmeldy (atidarsagene autotemcel)
Overview
Company
Orchard Therapeutics (Kyowa Kirin)
Overview
Modality
CGT
Overview
Target
ARSA (arylsulfatase A) gene
Overview
Indication
Pre-symptomatic and early-symptomatic metachromatic leukodystrophy
Overview
Phase
APPROVED
Overview
Status
APPROVED
Overview
Content status
Curated Core
Overview
Data Confidence
Data Confidence · High
Overview
Development Signal
Development Signal · Established
Overview
Approval status
FDA approved
Positioning
Key differentiator
Transduced microglia-like cells cross into the CNS and cross-correct neighboring cells — enzyme replacement cannot reach that compartment.
Positioning
Known limitation
Late-symptomatic disease has irreversible demyelination — timing, not biology, is the limit.
Positioning
Development positioning
No approved alternative; HSCT alone does not prevent late-infantile progression.
Technology
Vector
Lentiviral vector encoding human ARSA
MoA
Mechanism
Autologous CD34+ cells carrying a functional ARSA transgene engraft and differentiate; ARSA-secreting myeloid progeny including CNS microglia restore sulfatide degradation and halt demyelination.
MoA
Biomarker
ARSA enzyme activity in peripheral blood mononuclear cells, CSF sulfatide, vector copy number.
PK/PD
Half-life
Durable engraftment; ARSA activity restored within months of infusion
PK/PD
Species
ARSA activity, GMFM motor scores, DQ/IQ trajectory
PK/PD
Animal (cat.)
Human
PK/PD
Experiment
pharmacodynamic
Toxicology
Major finding
Busulfan conditioning toxicity — cytopenia, mucositis, infection, and fertility risk. Anti-ARSA antibodies and theoretical insertional oncogenesis require long-term follow-up.
Toxicology
CRS
N/A
Clinical
Safety signal
Busulfan conditioning toxicity — cytopenia, mucositis, infection, and fertility risk. Anti-ARSA antibodies and theoretical insertional oncogenesis require long-term follow-up.
Clinical
Result source
Orchard OTL-200 integrated analysis (NCT01560182)
Clinical
Program phase
APPROVED
Clinical
Trial activity
1 recruiting · 2 completed
Clinical
Trial ref
NCT01560182