[120] Cerebroside sulfate sulfatase (arylsulfatase A) from human urine

1972 ◽  
pp. 880-884 ◽  
Author(s):  
J.L. Breslow ◽  
H.R. Sloan
Keyword(s):  
Human Urine ◽  
Arylsulfatase A ◽  
Cerebroside Sulfate

scholarly journals Pseudoarylsulfatase-A Deficiency in the Neurologically Impaired Patient

1985 ◽  
Vol 12 (3) ◽  
pp. 274-277 ◽  
Author(s):  
Kevin Farrell ◽  
D.A. Applegarth ◽  
J.R. Toone ◽  
P.M. McLeod ◽  
A.V. Savage
Keyword(s):  
Nerve Biopsy ◽  
Specific Method ◽  
Arylsulfatase A ◽  
Neurologically Impaired ◽  
Loading Test ◽  
Cultured Fibroblasts ◽  
Cerebroside Sulfate ◽  
Neurological Findings ◽  
Impaired Patient ◽  
Metachromatic Leucodystrophy

ABSTRACT:The demonstration of low arylsulfatase-A (ASA) activity in leucocytes or fibroblasts is used often to establish the diagnosis of metachromatic leucodystrophy (MLD). However, low ASA activity is observed also in pseudo-ASA deficiency which may be as common as MLD. We report two patients with pseudo ASA deficiency who had abnormal neurological findings consistent with atypical MLD. Because the measurement of ASA activity is neither a sensitive nor specific method with which to establish a diagnosis of MLD, this diagnosis should be confirmed by nerve biopsy, measurement of urinary sulfatide or a cerebroside sulfate loading test, using cultured fibroblasts.

Preparation of the Cerebroside Sulfate Activator (CSAct or Saposin B) from Human Urine

1999 ◽  
Vol 68 (3) ◽  
pp. 391-403 ◽  
Author(s):  
Arvan L Fluharty ◽  
Claudio Lombardo ◽  
Andrew Louis ◽  
Richard L Stevens ◽  
Julian Whitelegge ◽  
...  
Keyword(s):  
Human Urine ◽  
Cerebroside Sulfate ◽  
Saposin B

scholarly journals Impaired Cerebroside Sulfate Hydrolysis in Fibroblasts of Sibs with “Pseudo” Arylsulfatase A Deficiency without Metachromatic Leukodystrophy

1983 ◽  
Vol 17 (9) ◽  
pp. 701-704 ◽  
Author(s):  
Stefan J Hreidarsson ◽  
George H Thomas ◽  
Hayato Kihara ◽  
Arvan L Fluharty ◽  
Edwin H Kolodny ◽  
...  
Keyword(s):  
Metachromatic Leukodystrophy ◽  
Arylsulfatase A ◽  
Cerebroside Sulfate

scholarly journals A STUDY OF ARYLSULFATASE A IN HUMAN URINE

1984 ◽  
Vol 75 (4) ◽  
pp. 672-680
Author(s):  
Akio Maru ◽  
Kimiyoshi Mitsuhashi ◽  
Masao Kosugi ◽  
Toshimori Seki ◽  
Tomohiko Koyanagi
Keyword(s):  
Human Urine ◽  
Arylsulfatase A

scholarly journals Prenatal Diagnosis of Metachromatic Leukodystrophy in a Family with Pseudo Arylsulfatase A Deficiency by the Cerebroside Sulfate Loading Test

1980 ◽  
Vol 14 (3) ◽  
pp. 224-227 ◽  
Author(s):  
Hayato Kihara ◽  
Chen-Kung Ho ◽  
Arvan L Fluharty ◽  
Katherine K Tsay ◽  
Patricia L Hartlage
Keyword(s):  
Prenatal Diagnosis ◽  
Metachromatic Leukodystrophy ◽  
Arylsulfatase A ◽  
Loading Test ◽  
Cerebroside Sulfate

A second form of arylsulfatase A in human urine

1973 ◽  
Vol 302 (2) ◽  
pp. 338-344 ◽  
Author(s):  
Richard L. Stevens ◽  
Michiyo Hartman ◽  
Arvan L. Fluharty ◽  
Hayato Kihara
Keyword(s):  
Human Urine ◽  
Arylsulfatase A

scholarly journals A novel mass spectrometric assay for the cerebroside sulfate activator protein (saposin B) and arylsulfatase A

2005 ◽  
Vol 46 (10) ◽  
pp. 2254-2264 ◽  
Author(s):  
Andrew J. Norris ◽  
Julian P. Whitelegge ◽  
Arman Yaghoubian ◽  
Jean-Rene Alattia ◽  
Gilbert G. Privé ◽  
...  
Keyword(s):  
Mass Spectrometric ◽  
Arylsulfatase A ◽  
Cerebroside Sulfate ◽  
Activator Protein ◽  
Saposin B
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