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Author Report for: Hofmann SL

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    Title: Intrathecal enzyme replacement therapy improves motor function and survival in a preclinical mouse model of infantile neuronal ceroid lipofuscinosis
    Lu JY, Nelvagal HR, Wang L, Birnbaum SG, Cooper JD, Hofmann SL
    Ref: Mol Genet Metab, 116:98, 2015 : PubMed

            

    Title: Intravenous high-dose enzyme replacement therapy with recombinant palmitoyl-protein thioesterase reduces visceral lysosomal storage and modestly prolongs survival in a preclinical mouse model of infantile neuronal ceroid lipofuscinosis
    Hu J, Lu JY, Wong AM, Hynan LS, Birnbaum SG, Yilmaz DS, Streit BM, Lenartowicz EM, Thompson TC and Hofmann SL <1 more author(s)>
    Ref: Mol Genet Metab, 107:213, 2012 : PubMed

            

    Title: Human recombinant palmitoyl-protein thioesterase-1 (PPT1) for preclinical evaluation of enzyme replacement therapy for infantile neuronal ceroid lipofuscinosis
    Lu JY, Hu J, Hofmann SL
    Ref: Mol Genet Metab, 99:374, 2010 : PubMed

            

    Title: A mutation in canine PPT1 causes early onset neuronal ceroid lipofuscinosis in a Dachshund
    Sanders DN, Farias FH, Johnson GS, Chiang V, Cook JR, O'Brien DP, Hofmann SL, Lu JY, Katz ML
    Ref: Mol Genet Metab, 100:349, 2010 : PubMed

            

    Title: Gene expression profiling in a mouse model of infantile neuronal ceroid lipofuscinosis reveals upregulation of immediate early genes and mediators of the inflammatory response
    Qiao X, Lu JY, Hofmann SL
    Ref: BMC Neurosci, 8:95, 2007 : PubMed

            

    Title: Functional biology of the neuronal ceroid lipofuscinoses (NCL) proteins
    Kyttala A, Lahtinen U, Braulke T, Hofmann SL
    Ref: Biochimica & Biophysica Acta, 1762:920, 2006 : PubMed

            

    Title: Thematic review series: lipid posttranslational modifications. Lysosomal metabolism of lipid-modified proteins
    Lu JY, Hofmann SL
    Ref: J Lipid Res, 47:1352, 2006 : PubMed

            

    Title: Progressively reduced synaptic vesicle pool size in cultured neurons derived from neuronal ceroid lipofuscinosis-1 knockout mice
    Virmani T, Gupta P, Liu X, Kavalali ET, Hofmann SL
    Ref: Neurobiol Dis, 20:314, 2005 : PubMed

            

    Title: The crystal structure of palmitoyl protein thioesterase-2 (PPT2) reveals the basis for divergent substrate specificities of the two lysosomal thioesterases, PPT1 and PPT2
    Calero G, Gupta P, Nonato MC, Tandel S, Biehl ER, Hofmann SL, Clardy J
    Ref: Journal of Biological Chemistry, 278:37957, 2003 : PubMed

            

    Title: Early changes in gene expression in two models of Batten disease
    Elshatory Y, Brooks AI, Chattopadhyay S, Curran TM, Gupta P, Ramalingam V, Hofmann SL, Pearce DA
    Ref: FEBS Letters, 538:207, 2003 : PubMed

            

    Title: Disruption of PPT2 in mice causes an unusual lysosomal storage disorder with neurovisceral features
    Gupta P, Soyombo AA, Shelton JM, Wilkofsky IG, Wisniewski KE, Richardson JA, Hofmann SL
    Ref: Proc Natl Acad Sci U S A, 100:12325, 2003 : PubMed

            

    Title: Biochemical analysis of mutations in palmitoyl-protein thioesterase causing infantile and late-onset forms of neuronal ceroid lipofuscinosis
    Das AK, Lu JY, Hofmann SL
    Ref: Hum Mol Genet, 10:1431, 2001 : PubMed

            

    Title: Disruption of PPT1 or PPT2 causes neuronal ceroid lipofuscinosis in knockout mice
    Gupta P, Soyombo AA, Atashband A, Wisniewski KE, Shelton JM, Richardson JA, Hammer RE, Hofmann SL
    Ref: Proc Natl Acad Sci U S A, 98:13566, 2001 : PubMed

            

    Title: The crystal structure of palmitoyl protein thioesterase 1 and the molecular basis of infantile neuronal ceroid lipofuscinosis
    Bellizzi JJ, 3rd, Widom J, Kemp C, Lu JY, Das AK, Hofmann SL, Clardy J
    Ref: Proc Natl Acad Sci U S A, 97:4573, 2000 : PubMed

            

    Title: Structural basis for the insensitivity of a serine enzyme (palmitoyl-protein thioesterase) to phenylmethylsulfonyl fluoride
    Das AK, Bellizzi JJ, 3rd, Tandel S, Biehl E, Clardy J, Hofmann SL
    Ref: Journal of Biological Chemistry, 275:23847, 2000 : PubMed

            

    Title: Identification of three novel mutations of the palmitoyl-protein thioesterase-1 (PPT1) gene in children with neuronal ceroid-lipofuscinosis
    Waliany S, Das AK, Gaben A, Wisniewski KE, Hofmann SL
    Ref: Hum Mutat, 15:206, 2000 : PubMed

            

    Title: Structure of the human palmitoyl-protein thioesterase-2 gene (PPT2) in the major histocompatibility complex on chromosome 6p21.3
    Soyombo AA, Yi W, Hofmann SL
    Ref: Genomics, 56:208, 1999 : PubMed

            

    Title: Molecular genetics of palmitoyl-protein thioesterase deficiency in the U.S
    Das AK, Becerra CH, Yi W, Lu JY, Siakotos AN, Wisniewski KE, Hofmann SL
    Ref: Journal of Clinical Investigation, 102:361, 1998 : PubMed

            

    Title: Mutations in the palmitoyl-protein thioesterase gene (PPT; CLN1) causing juvenile neuronal ceroid lipofuscinosis with granular osmiophilic deposits
    Mitchison HM, Hofmann SL, Becerra CH, Munroe PB, Lake BD, Crow YJ, Stephenson JB, Williams RE, Hofman IL and O'Rawe AM <7 more author(s)>
    Ref: Hum Mol Genet, 7:291, 1998 : PubMed

            

    Title: Molecular cloning and expression of palmitoyl-protein thioesterase 2 (PPT2), a homolog of lysosomal palmitoyl-protein thioesterase with a distinct substrate specificity
    Soyombo AA, Hofmann SL
    Ref: Journal of Biological Chemistry, 272:27456, 1997 : PubMed

            

    Title: Lipid thioesters derived from acylated proteins accumulate in infantile neuronal ceroid lipofuscinosis: correction of the defect in lymphoblasts by recombinant palmitoyl-protein thioesterase
    Lu JY, Verkruyse LA, Hofmann SL
    Ref: Proc Natl Acad Sci U S A, 93:10046, 1996 : PubMed

            

    Title: cDNA and genomic cloning of human palmitoyl-protein thioesterase (PPT), the enzyme defective in infantile neuronal ceroid lipofuscinosis
    Schriner JE, Yi W, Hofmann SL
    Ref: Genomics, 34:317, 1996 : PubMed

            

    Title: Lysosomal targeting of palmitoyl-protein thioesterase
    Verkruyse LA, Hofmann SL
    Ref: Journal of Biological Chemistry, 271:15831, 1996 : PubMed

            

    Title: Mutations in the palmitoyl protein thioesterase gene causing infantile neuronal ceroid lipofuscinosis
    Vesa J, Hellsten E, Verkruyse LA, Camp LA, Rapola J, Santavuori P, Hofmann SL, Peltonen L
    Ref: Nature, 376:584, 1995 : PubMed

            

    Title: Molecular cloning and expression of palmitoyl-protein thioesterase
    Camp LA, Verkruyse LA, Afendis SJ, Slaughter CA, Hofmann SL
    Ref: Journal of Biological Chemistry, 269:23212, 1994 : PubMed

            


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