PURIFIED ARYLSULFATASE A AND COMPOSITIONS THEREOF

1 - 81 . (canceled)

82 . A composition for treating metachromatic leukodystrophy (MLD), comprising purified recombinant human arylsulfatase A (rhASA) protein having an amino acid sequence at least 70% identical to SEQ ID NO: 1, wherein the purified rhASA protein is characterized by one or more proteoglycan species selected from:

i. 15% to 25% neutral recombinant ASA protein (neutral ASA protein),

ii. 35% to 45% sialylated recombinant ASA protein (sialic acid ASA protein),

iii. 23% to 33% mannose-6-phosphated recombinant ASA protein (M6P ASA protein),

iv. 1% to 10% N-acetyl-glucosamine mannose-6-phosphated recombinant ASA protein (capped M6P ASA protein), and

v. 5% to 15% hybrid recombinant ASA protein (hybrid ASA protein).

83 . The composition for use of claim 82 , wherein the recombinant ASA contains less than about 150 ng/mg Host Cell Protein (HCP).

84 . The composition of claim 82 , wherein at least about 23% of the total purified recombinant ASA protein corresponds to mannose-6-phosphated recombinant ASA protein (M6P ASA protein).

85 . The composition of claim 82 , wherein the total purified rhASA protein is present in a concentration of about 20 mg/mL to about 45 mg/mL.

86 . The composition of claim 82 , wherein the total purified rhASA protein is present in a concentration of about 25 mg/mL to about 34 mg/mL or about 28 mg/mL to about 32 mg/mL.

87 . The composition of claim 82 , wherein the purified rhASA protein has a specific activity of about 50 to about 130 U/mL.

88 . The composition of claim 87 , wherein the purified rhASA protein has a specific activity of about 70 to about 100 U/mg.

89 . The composition of claim 82 , wherein the purified rhASA protein contains less than about 140 ng/mg Host Cell Protein (HCP).

90 . The composition of claim 82 , wherein the purified rhASA protein contains less than about 100 pg/mg Host Cell DNA (HCD).

91 . The composition of claim 82 , comprising about 0.001% to about 0.01% polysorbate-20 (P20).

92 . A formulation for treating metachromatic leukodystrophy (MLD), comprising the composition of claim 91 and a physiologically acceptable carrier.

93 . The formulation of claim 92 , wherein the formulation is suitable for intravenous administration.

94 . The formulation of claim 92 , wherein the formulation is suitable for intrathecal administration.

95 . The formulation of claim 92 , wherein the formulation is suitable for subcutaneous administration.

96 . A method of treating metachromatic leukodystrophy (MLD), the method comprising administering to a subject in need thereof a therapeutically effective dose of a purified recombinant human arylsulfatase A (rhASA) protein,

wherein the purified rhASA protein is characterized by a proteoglycan map comprising one or more proteoglycan species selected from:

i. 15% to 25% neutral recombinant ASA protein (neutral ASA protein),

ii. 35% to 45% sialylated recombinant ASA protein (sialic acid ASA protein),

iii. 23% to 33% mannose-6-phosphated recombinant ASA protein (M6P ASA protein),

iv. 1% to 10% N-acetyl-glucosamine mannose-6-phosphated recombinant ASA protein (capped M6P ASA protein), and

v. 5% to 15% hybrid recombinant ASA protein (hybrid ASA protein).

97 . A method of treating metachromatic leukodystrophy (MLD), the method comprising administering a recombinant arylsulfatase A (ASA) enzyme for use in treating metachromatic leukodystrophy (MLD) in a subject characterized as having a baseline of less than level 4 by Gross Motor Function Classification (GMFC), wherein the purified rhASA protein is characterized by a proteoglycan map comprising one or more proteoglycan species selected from:

i. 15% to 25% neutral recombinant ASA protein (neutral ASA protein),

ii. 35% to 45% sialylated recombinant ASA protein (sialic acid ASA protein),

iii. 23% to 33% mannose-6-phosphated recombinant ASA protein (M6P ASA protein),

iv. 1% to 10% N-acetyl-glucosamine mannose-6-phosphated recombinant ASA protein (capped M6P ASA protein), and

v. 5% to 15% hybrid recombinant ASA protein (hybrid ASA protein), and

wherein the recombinant ASA enzyme is for intrathecal administration to the subject at a therapeutically effective dose.

98 . The method of claim 97 , wherein the method comprises administering a recombinant arylsulfatase A (ASA) enzyme for use in treating metachromatic leukodystrophy (MLD) in a subject characterized as having a baseline of level 1-4 by Gross Motor Function Classification (GMFC), wherein the recombinant ASA enzyme is for intrathecal administration at a therapeutically effective dose, and wherein the recombinant ASA enzyme is characterized by a proteoglycan map comprising one or more proteoglycan species selected from:

i. 15% to 25% neutral recombinant ASA protein (neutral ASA protein),

ii. 35% to 45% sialylated recombinant ASA protein (sialic acid ASA protein),

iii. 23% to 33% mannose-6-phosphated recombinant ASA protein (M6P ASA protein),

iv. 1% to 10% N-acetyl-glucosamine mannose-6-phosphated recombinant ASA protein (capped M6P ASA protein), and

v. 5% to 15% hybrid recombinant ASA protein (hybrid ASA protein).

99 . The method of claim 96 , wherein the purified rhASA protein is characterized by a proteoglycan map comprising:

i. about 18% to about 22% neutral ASA protein,

ii. about 37% to about 41% sialic acid ASA protein,

iii. about 26% to about 29% M6P ASA protein,

iv. about 4% to about 6% capped M6P ASA protein, and

v. about 7% to about 9% hybrid ASA protein.

100 . The method of claim 99 , wherein the subject has late infantile MLD and wherein the subject is less than 18 months old.

101 . The method of claim 96 , wherein the rhASA protein has an amino acid sequence that is at least 70% identical to SEQ ID NO: 1.