A 1-deoxyinojirimycin (DNJ) derivative comprising a compound of the formula: ** Formula ** where R 1 is H or a linear or branched alkyl, cycloalkyl, alkenyl, alkoxyalkyl or aminoalkyl containing 1-12 carbon atoms, a aryl, alkylaryl, heteroaryl or heteroarylalkyl containing 5-12 ring atoms, where R1 is optionally substituted with one or more -OH, -COOH, -Cl, -F, -CF3, -OCF3, -OC (>； = O) N- (alkyl) 2； R2 is H； a linear or branched alkyl, cycloalkyl, alkenyl, alkylaryl or alkoxyalkyl, containing 1-9 carbon atoms or aryl containing 5-12 carbon atoms, wherein R2 is optionally substituted with -OH, -COOH, -CF3, - OCF3 or a heterocyclic ring； wherein at least one of R1 and R2 is not H； or a pharmaceutically acceptable salt thereof, for use in the treatment of Pompe disease, wherein the DNJ derivative is used to increase the activity of a lysosomal α-glucosidase (Gaa) enzyme in a cell, at a concentration of or below the IC50 value for intestinal Gaa inhibition； wherein said DNJ derivative must be administered as a monotherapy in an individual who has Pompe disease； and wherein the DNJ derivative is not 1-deoxinojirimycin or α-homonojirimycin； and wherein the DNJ derivative is administered in an amount of 5 mg to 75 mg daily.Un derivado de 1-desoxinojirimicina (DNJ) que comprende un compuesto de la fórmula:**Fórmula** donde R1 es H o un alquilo, cicloalquilo, alquenilo, alcoxialquilo o aminoalquilo lineal o ramificado que contiene 1 - 12 átomos de carbono, un arilo, alquilarilo, heteroarilo o heteroarilalquilo que contiene 5 - 12 átomos de anillo, donde R1 está opcionalmente sustituido con uno o más -OH, -COOH, -Cl, -F,-CF3, -OCF3, -O-C(>；=O)N-(alquilo)2； R2 es H； un alquilo, cicloalquilo, alquenilo, alquilarilo o alcoxialquilo lineal o ramificado, que contiene 1-9 átomos de carbono o arilo que contiene 5 - 12 átomos de carbono, en donde R2 está opcionalmente sustituido con -OH, -COOH, -CF3, -OCF3 o un anillo heterocíclico； en donde al menos uno de R1 y R2 no es H； o un
