Hunters disease is caused by?
ANSWER: (B) Iduronate sulfataseREF: Harper 27th ed chapter 47See APPENDIX-17 for "LYSOSOMAL STORAGE DISORDERS" APPENDIX - 17Lysosomal Storage Disorders DiseaseDeficiencyInheritance(1) Lipid Storage Disorder(Sphingolipidoses)GM1 gangliosidosesBeta galactosidaseARGM2 gangliosidosesTay-sach diseaseBeta hexosaminidase -A & BARSandhoff diseaseBeta hexosaminidase -A & BARGaucher disease (most common LSD)GlucocerebrosidaseARNiemann-PickdiseaseType-A&BSphingomyelinaseARType- C&DNPC1 or NPC2 proteinsARFabry diseaseAlpha- galactosidase-AXRFarber's diseaseCeramidaseARKrabbe diseaseGalactosylceramidaseARMetachromatic leukodystrophy(MLD)Arylsulfatase AARWolman's diseaseacid lipaseAR(2) Mucopoly SaccharidosesMPS I- Hurler syndrome MPS II- Hunter syndromea-L-iduronidase Iduronate sulfataseARXRMPS IIISanfilippo syndrome AHeparan sulfamidaseARSanfilippo syndrome BN-acetylglucosaminidase Sanfilippo syndrome CAcetyl-CoA:alpha-glucosaminideacetyltransferase Sanfilippo syndrome DN-acetylglucosamine 6- sulfatase MPS IVMorquio syndrome AGalactose-6-sulfate sulfataseARMorquio syndrome BBe ta-galactosidase MPS VI- Maroteaux-Lamy syndromeN-acetyigalactosamine-4-sulfataseARMPS VII- Sly syndrome/2-glucuronidaseARMPS IX- Natowicz syndromeHyaluronidaseAR(3) GlycoproteinosisMucolipidosisSialidosis (mucolipidosis I)stalidaseARMucolipidosis IIN-acetylglucosamine-1- phosphotransferaseMucolipidosis III(pseudo-Hurlerpolydystrophy)N-acetylglucosamine-1-phosphotransferaseFucosidosis alpha-l-fucosidaseARMannosidosis Alpha & Beta-mannosidosisARNOTE: All except Fabry's and Hunter's disease (x linked recessive) are autosomal recessive.
a-L-iduronidase
Iduronate sulfatase
Aryl Sulfatase
a-glucuronidase
Phospholipids, Glycolipids and their disorders