AR123725A1 - COMPOSITIONS AND METHODS FOR THE TREATMENT OF FABRY DISEASE - Google Patents

COMPOSITIONS AND METHODS FOR THE TREATMENT OF FABRY DISEASE

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Publication number
AR123725A1
AR123725A1 ARP210102796A ARP210102796A AR123725A1 AR 123725 A1 AR123725 A1 AR 123725A1 AR P210102796 A ARP210102796 A AR P210102796A AR P210102796 A ARP210102796 A AR P210102796A AR 123725 A1 AR123725 A1 AR 123725A1
Authority
AR
Argentina
Prior art keywords
hgla
seq
expression
raav
coding sequence
Prior art date
Application number
ARP210102796A
Other languages
Spanish (es)
Inventor
Juliette Hordeaux
Original Assignee
Univ Pennsylvania
Priority date (The priority date is an assumption and is not a legal conclusion. Google has not performed a legal analysis and makes no representation as to the accuracy of the date listed.)
Filing date
Publication date
Application filed by Univ Pennsylvania filed Critical Univ Pennsylvania
Publication of AR123725A1 publication Critical patent/AR123725A1/en

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  • Micro-Organisms Or Cultivation Processes Thereof (AREA)
  • Medicines That Contain Protein Lipid Enzymes And Other Medicines (AREA)
  • Medicines Containing Material From Animals Or Micro-Organisms (AREA)

Abstract

Se proporcionan en el presente documento secuencias de polinucleótidos que codifican la a-galactosidasa A humana funcional (hGLA) y casetes de expresión que contienen estas secuencias codificantes. También se proporcionan vectores, tales como vectores de virus adenoasociados recombinantes (rAAV) que tienen genomas vectoriales que incluyen una secuencia codificante de hGLA unida operativamente a una o más secuencias reguladoras. Además, se proporcionan composiciones que contienen estos casetes de expresión y rAAV, así como métodos para el uso de estas composiciones para el tratamiento de la enfermedad de Fabry. Reivindicación 1: Un AAV recombinante (rAAV) que comprende una cápside AAVhu68 que lleva empaquetada un genoma vectorial, caracterizado porque el genoma vectorial comprende una secuencia de codificación para una a-galactosidasa A humana (hGLA) funcional y secuencias reguladoras que dirigen la expresión de la hGLA en una célula objetivo, donde la secuencia de codificación comprende los nucleótidos 94 a 1287 de la SEQ ID nº 4 o una secuencia al menos 85% idéntica a esta, y donde la hGLA tiene un residuo cisteínico en la posición 233 y/o en la posición 359 según la numeración de residuos de aminoácidos de la SEQ ID nº 2. Reivindicación 12: Un casete de expresión que comprende una secuencia de codificación de ácido nucleico que codifica una a-galactosidasa A humana (hGLA) funcional y una o más secuencias reguladoras que dirigen la expresión de la hGLA en una célula objetivo que contiene el casete de expresión, caracterizado porque la secuencia de ácidos nucleicos comprende los nucleótidos 94 a 1287 de la SEQ ID nº 4 o una secuencia al menos 85% idéntica a esta, y donde la hGLA tiene un residuo cisteínico en la posición 233 y/o en la posición 359 según la numeración de residuos de aminoácidos de la SEQ ID nº 2 o la SEQ ID nº 7. Reivindicación 26: Una célula hospedadora que comprende el casete de expresión según una cualquiera de las reivindicaciones 12 a 24 o el plásmido según la reivindicación 25. Reivindicación 27: Una composición farmacéutica que comprende el rAAV según una cualquiera de las reivindicaciones 1 a 11 o el casete de expresión según una cualquiera de las reivindicaciones 12 a 24, y un excipiente farmacéuticamente aceptable.Provided herein are polynucleotide sequences encoding functional human α-galactosidase A (hGLA) and expression cassettes containing these coding sequences. Vectors are also provided, such as recombinant adeno-associated virus (rAAV) vectors having vector genomes that include an hGLA coding sequence operatively linked to one or more regulatory sequences. In addition, compositions containing these expression cassettes and rAAV are provided, as well as methods of using these compositions for the treatment of Fabry disease. Claim 1: A recombinant AAV (rAAV) comprising an AAVhu68 capsid packaged with a vector genome, characterized in that the vector genome comprises a coding sequence for a functional human α-galactosidase A (hGLA) and regulatory sequences directing the expression of hGLA in a target cell, where the coding sequence comprises nucleotides 94 to 1287 of SEQ ID NO: 4 or a sequence at least 85% identical thereto, and where the hGLA has a cysteine residue at position 233 and/or at position 359 according to the amino acid residue numbering of SEQ ID No. 2. Claim 12: An expression cassette comprising a nucleic acid coding sequence encoding a functional human α-galactosidase A (hGLA) and one or more regulatory sequences directing the expression of hGLA in a target cell containing the expression cassette, characterized in that the nucleic acid sequence comprises the nucleotides 94 to 1287 of SEQ ID No. 4 or a sequence at least 85% identical thereto, and where hGLA has a cysteine residue at position 233 and/or position 359 according to the amino acid residue numbering of SEQ ID No. 2 or SEQ ID No. 7. Claim 26: A host cell comprising the expression cassette according to any one of claims 12 to 24 or the plasmid according to claim 25. Claim 27: A pharmaceutical composition comprising the rAAV according to a any one of claims 1 to 11 or the expression cassette according to any one of claims 12 to 24, and a pharmaceutically acceptable excipient.

ARP210102796A 2020-10-09 2021-10-07 COMPOSITIONS AND METHODS FOR THE TREATMENT OF FABRY DISEASE AR123725A1 (en)

Applications Claiming Priority (1)

Application Number Priority Date Filing Date Title
US202063089850P 2020-10-09 2020-10-09

Publications (1)

Publication Number Publication Date
AR123725A1 true AR123725A1 (en) 2023-01-04

Family

ID=85131234

Family Applications (1)

Application Number Title Priority Date Filing Date
ARP210102796A AR123725A1 (en) 2020-10-09 2021-10-07 COMPOSITIONS AND METHODS FOR THE TREATMENT OF FABRY DISEASE

Country Status (1)

Country Link
AR (1) AR123725A1 (en)

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