Merry B. Passage

Merry B. Passage

Division Of Medical Genetics, Department Of Pediatrics, La Biomed At Harbor-Ucla, 1124 W. Carson Street, Building E-4, Torrance, Ca 90502, Usa

Direct Impact

Concepts for which Merry B Passage has direct influence:

immune tolerance
lysosomal storage
enzyme replacement therapy
canine mucopolysaccharidosis
sulfate transport
cultured cells
continuous infusion

External impact

Concepts related to the work of other authors for which Merry B Passage has influence:

lysosomal storage
immune response
anti-hgh antibodies
replacement therapy
adenine nucleotide translocase
transport mechanism
chromosomal localization

Prominent publications by Merry B. Passage

KOL-Index: 30 Immune responses can interfere with the effective use of therapeutic proteins to treat genetic deficiencies and have been challenging to manage. To address this problem, we adapted and studied methods of immune tolerance used in canine organ transplantation research to soluble protein therapeutics. A tolerization regimen was developed that prevents a strong antibody response to the enzyme ...
Known for
Lysosomal Storage Disorder Mucopolysaccharidosis | Reduced Immune Response | Azathioprine | Limited 60-Day
KOL-Index: 19 Intrathecal (IT) recombinant human α-l-iduronidase (rhIDU) has been shown to reduce mean brain glycosaminoglycans (GAGs) to normal levels in mucopolysaccharidosis I (MPS I) dogs. In this study, we examined storage in neuroanatomical regions of the MPS I dog brain, including frontal lobe, cerebellum, basal ganglia, thalamus, hippocampal formation, and brainstem, to determine the response of ...
Known for
Gag | Lobe Brain | Regions Mps
KOL-Index: 19 A 9-year-old mentally retarded girl with multiple congenital anomalies was found to carry a balanced 13/14 Robertsonian translocation [45,XX,t(13q14q)] which was also present in her father. Her mother carried a balanced reciprocal translocation between chromosomes 1 and 14 [46,XX,t(1;14) (q32;q32)]. Both of her parents were phenotypically normal. Molecular studies were carried out to ...
Known for
Balanced Reciprocal Translocation | Maternal Paternal Alleles | 13 D13s37 Loci | Abnormal Phenotypically
KOL-Index: 17 Mucopolysaccharidosis type I (MPS I) is a lysosomal storage disease caused by loss of activity of α-l-iduronidase and attendant accumulation of the glycosaminoglycans dermatan sulfate and heparan sulfate. Current treatments are suboptimal and do not address residual disease including corneal clouding, skeletal deformities, valvular heart disease, and cognitive impairment. We treated neonatal ...
Known for
Treated Neonatal | Intravenous Intrathecal | Mps Lysosomal | Recombinant Iduronidase
KOL-Index: 16 The expression of mouseZfx, Rps4, Ube1x, andXist was evaluated in hamstermouse somatic cell hybrids containing either an active or an inactive mouse X chromosome using polymerase chain reaction of reverse transcribed RNA (RT-PCR). The results showed thatZfx, Rps4, andUbe1x are expressed exclusively from the active mouse X, whileXist is expressed exclusively from the inactive X. These ...
Known for
Inactive Mouse Chromosome | Hamstermouse Somatic Hybrids | Reverse Transcribed Rna | F1 Females
KOL-Index: 16 Mucopolysaccharidoses (MPSs) are lysosomal storage diseases caused by a deficit in the enzymes needed for glycosaminoglycan (GAG) degradation. Enzyme replacement therapy with recombinant human alpha-L-iduronidase successfully reduces lysosomal storage in canines and humans with iduronidase-deficient MPS I, but therapy usually also induces antibodies specific for the recombinant enzyme that ...
Known for
Mucopolysaccharidoses | Immune-Tolerized Canines | Tolerized Mps | Increased Tissue Enzyme
KOL-Index: 16 Using thiol blocking agents, we examined the role of sulphydryl groups for function of the lysosomal sulphate transport system. Monothiol binding reagents, p-hydroxymercuribenzoic acid (p-HMB) and p-chloromercuribenzene sulphonic acid (p-CMBS), dithiol binding reagents such as CuCl2, the alkylating agent, N-ethylmaleimide (NEM), and NADH all inhibited lysosomal sulphate transport. The ...
Known for
Transport Inhibitory | Binding Reagents | Sulphydryl Affinity | Role Sulphate
KOL-Index: 15 The relative order of 11 loci in the distal half of the short arm of the human X chromosome was examined using a panel of somatic cell hybrids containing structurally rearranged X chromosomes. The results show that the gene for phosphoribosylpyrophosphate synthetase 2 (PRPS2) is located betweenZFX (zinc finger protein, X-linked) andSTS (steroid sulfatase). The results also confirm the ...
Known for
-Inactivation Whereaspola Undergoes Inactivation | Loci Distal | Phosphoribosylpyrophosphate Synthetase | Structurally Rearranged Chromosomes
KOL-Index: 15 BACKGROUND: Mucopolysaccharidosis I is a lysosomal storage disease caused by a deficiency of the enzyme alpha-L-iduronidase. We evaluated the effect of enzyme-replacement therapy with recombinant human alpha-L-iduronidase in patients with this disorder. METHODS: We treated 10 patients with mucopolysaccharidosis I (age, 5 to 22 years) with recombinant human alpha-L-iduronidase at a dose of ...
Known for
Therapy Recombinant | Motion Measurements | Weight Increased | Evaluated Enzyme

Division of Medical Genetics, Department of Pediatrics, LA Biomed at Harbor-UCLA, 1124 W. Carson Street, Building E-4, Torrance, CA 90502, USA

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