Dr Wei-Li Di

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Personal Profile

Name: Wei-Li Di Email: w.di@ucl.ac.uk
Title: Dr Tel:
Department: ICH - Immunobiology Unit Fax: 020 78138494
Position: Lecturer Address: 30 Guilford Street, London, London, WC1N 1EH
Research Domain: Basic Life Sciences, Personalised Medicine, Populations & Lifelong Health Web Page:  

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Research Description

My work is in two major research areas: i) the involvement of SPINK5 and its down-stream targets including kallikreins and protease activated receptor 2 (PAR2) in skin barrier function and atopic dermatitis and ii) gene therapy for Netherton syndrome.

Research Activities

Pathogenesis and therapeutic strategies for skin diseases with impaired skin barrier function

Education Description

UCL Collaborators

External Collaborators

Publications

    2013

    • Scott CA, Rajpopat S, Di W-L (2013). Harlequin ichthyosis: ABCA12 mutations underlie defective lipid transport, reduced protease regulation and skin-barrier dysfunction. Cell and Tissue Research, 351(2), 281 - 288. doi:10.1007/s00441-012-1474-9
    • Syed F, Abdul-Wahab A, Georgiadis C, Petrova A, Ghani S, Thrasher A, McGrath J, Di W-L, Qasim W (2013). Lentiviral-mediated COL7A1 gene modified autologous cell therapy for Recessive Dystrophic Epidermolysis Bullosa (RDEB).
    • Di W, Ghani S, Almarza D, Chen I, Mellerio J, Bernadis C, Harper J, Thrasher A, Farzaneh F, Qasim W (2013). Production of gene engineered epithelia sheets for first-in-man use.
    • Nowak K, Thrasher AJ, Di WL, Burns SO (2013). Keratinocytes express functional common gamma chain.
    • Zhu Y, Harper J, O'Shaughnessy R, Di W (2013). Kallikrein 5 up-regulation in epidermal barrier dysfunction and atopic dermatitis pathogenesis.
    • Naeem A, Zhu Y, Harper J, Di W, O'Shaughnessy R (2013). Akt-1 mediates nuclear lamin degradation in keratinocytes and the expression of Cathepsin H a novel epidermal protease involved in profilaggrin processing.
    • Georgiadis C, Semenova E, South AP, Thrasher A, McGrath JA, Di W-L, Qasim W (2013). Development of Lentiviral Gene Therapy for Type VII Collagen Deficient Epidermolysis Bullosa.
    • Ghani S, Di W-L, Semenova E, Chan L, Mellerio J, McGrath J, Harper J, Thrasher A, Farzaneh F, Qasim W (2013). Production of gene modified epithelial sheets for phase 1 clinical trial for Netherton Syndrome.
    • Di WL, Mellerio JE, Bernadis C, Harper J, Abdul-Wahab A, Ghani S, Martinez-Queipo M, Hara H, McNicol AM, McGrath J, Thrasher AJ, Qasim W (2013). Phase I study protocol for ex-vivo lentiviral gene therapy for the inherited skin disease, Netherton Syndrome.. Hum Gene Ther Clin Dev, , - . doi:10.1089/hum.2013.195
    • Di WL, Mellerio JE, Bernadis C, Harper J, Abdul-Wahab A, Ghani S, Chan L, Martinez-Queipo M, Hara H, McNicol AM, Farzaneh F, McGrath J, Thrasher A, Qasim W (2013). Phase I study protocol for ex vivo lentiviral gene therapy for the inherited skin disease, netherton syndrome.. Hum Gene Ther Clin Dev, 24(4), 182 - 190. doi:10.1089/humc.2013.195
    • Nowak K, Thrasher A, Di WL, Burns S (2013). The role of the common gamma chain in keratinocytes.

    2012

    • Ghani S, Di W-L, Semenova E, Chan L, Farzaneh F, Qasim W (2012). Clinical Grade Lentiviral Vector Production for the Inherited Skin Disease, Netherton Syndrome.
    • Georgiadis C, Thrasher A, South AP, McGrath J, Qasim W, Di W (2012). Development of lentiviral gene therapy for type VII collagen deficient epidermolysis bullosa.
    • Xiang NN, Di WL (2012). Therapeutic interventions for Netherton Syndrome.
    • Zhu Y, O'Shaughnessy R, Harper J, Di W (2012). Up-regulated expression of Kallikrein 5 in atopic dermatitis.
    • Di WL, Semenova E, Larcher F, Del Rio M, Harper JI, Thrasher AJ, Qasim W (2012). Human involucrin promoter mediates repression-resistant and compartment-specific LEKTI expression.. Hum Gene Ther, 23(1), 83 - 90. doi:10.1089/hum.2011.091
    • Georgiadis C, Semenova K, McGrath J, Di W, Qasim W (2012). Development of lentiviral gene therapy for type VII collagen deficient epidermolysis bullosa.
    • Semenova E, Ghani S, Chan L, Farzaneh F, Larcher F, Harper J, Thrasher A, Qasim W, Di WL (2012). Clinical grade lentiviral vector production for inherited skin disease.
    • Bennett K, Heywood W, Di WL, Harper J, Clayman GL, Jayakumar A, Callard R, Mills K (2012). The identification of a new role for LEKTI in the skin: The use of protein 'bait' arrays to detect defective trafficking of dermcidin in the skin of patients with Netherton syndrome.. J Proteomics, 75(13), 3925 - 3937. doi:10.1016/j.jprot.2012.04.045
    • Scott CA, Rajpopat S, Di WL (2012). Harlequin ichthyosis: ABCA12 mutations underlie defective lipid transport, reduced protease regulation and skin barrier dysfunction.

    2011

    • Di WL, Larcher F, Semenova E, Talbot GE, Harper JI, Del Rio M, Thrasher AJ, Qasim W (2011). Ex-vivo Gene Therapy Restores LEKTI Activity and Corrects the Architecture of Netherton Syndrome-derived Skin Grafts. MOL THER, 19(2), 408 - 416. doi:10.1038/mt.2010.201
    • Blaydon DC, Biancheri P, Di WL, Plagnol V, Cabral RM, Brooke MA, van Heel DA, Ruschendorf F, Toynbee M, Walne A, O'Toole EA, Martin JE, Lindley K, Vulliamy T, Abrams DJ, MacDonald TT, Harper JI, Kelsell DP (2011). Inflammatory skin and bowel disease linked to ADAM17 deletion.. N Engl J Med, 365(16), 1502 - 1508. doi:10.1056/NEJMoa1100721
    • Kelsell DP, Blaydon DC, Biancheri P, Di W, Plagnol V, Cabral RM, MacDonald TT, Harper JI (2011). Next-generation sequencing identifies a homozygous deletion mutation in an ADAM gene underlying autosomal recessive inflammatory skin and bowel disease..

    2010

    • Qasim W, Semenova E, Thrasher A, Larcher F, Di W (2010). Preclinical modelling of ex-vivo skin gene therapy for Netherton syndrome.
    • Semenova E, Qasim W, Larcher F, Talbot G, Thrasher AJ, Harper JI, Di WL (2010). Translational development of ex-vivo gene therapy for Netherton syndrome.
    • Semenova E, Larcher F, Talbot G, Harper JI, Thrasher AJ, Di WI, Qasim W (2010). Lentiviral Mediated LEKTI Expression Reverses the Netherton Syndrome Phenotype.
    • Wu XL, Zhu YN, Harper J, Callard R, Di WL (2010). Hyper-activation of kallikrein 5 does not up-regulate interleukin 8 expression in Netherton syndrome.
    • Bennett K, Callard R, Heywood W, Harper J, Jayakumar A, Clayman GL, Di WL, Mills K (2010). New role for LEKTI in skin barrier formation: label-free quantitative proteomic identification of caspase 14 as a novel target for the protease inhibitor LEKTI.. J Proteome Res, 9(8), 4289 - 4294. doi:10.1021/pr1003467

    2009

    • Qasim W, Talbot G, Larcher F, Harper J, Thrasher A, Di WL (2009). Development of Lentiviral Therapy for Netherton Syndrome.
    • Di WL, Hennekam RC, Callard RE, Harper JI (2009). A heterozygous null mutation combined with the G1258A.
    • Di WL, Hennekam RC, Callard RE, Harper JI (2009). A heterozygous null mutation combined with the G1258A polymorphism of SPINK5 causes impaired LEKTI function and abnormal expression of skin barrier proteins. British Journal of Dermatology, 161(2), 404 - 412.

    2008

    • Su Z, Hoey S, Jayakody S, Callard R, Harper J, Di W (2008). Over-expression of kallikrein 5 desensitises protease activated receptor 2 in keratinocytes - a possible pathomechanism of skin barrier dysfunction of AD.
    • Talbot GE, Qasim W, Thrasher AJ, Harper JI, Di WL (2008). Reproducible and stable transgene expression towards ex vivo lentiviral gene therapy for Netherton syndrome.
    • Talbot GE, Qasim W, Thrasher AJ, Harper JI, Di WL (2008). Reproducible and stable transgene expression towards ex vivo lentiviral gene therapy for Netherton Syndrome.
    • Bennett K, Mills K, Collard R, Harper J, Di W (2008). The identification of LEKTI targets using a novel proteomic approach.

    2007

    • Bennett K, Mills K, Callard R, Harper J, Di WL (2007). A proteomic approach to identify targets of LEKTI - a protein implicated in the barrier function of the skin.

    2006

    • Di W, Ong C, Callard RE, Hennekam RC, Harper JI (2006). Netherton's syndrome in a patient with a heterozygous mutation and homozygous G1258A polymorphism of SPINK5 (serine protease inhibitor, Kazal-type 5).

    2005

    • Aasen T, Di WL, Kelsell DP (2005). Elucidation of connexin31 as a regulator of epidermal differentiation and tissue homeostasis.
    • Ong C, Di WL, O'Toole EA, Green A, Callard R, Harper J (2005). LEKTI is required for skin barrier integrity and acts via the PAR-2 signalling pathway.
    • Aasen T, Unsworth H, Di W, Kelsell DP (2005). The gap junction protein connexin 31 regulates key aspects of cell differentiation.
    • Di W, Gu Y, Common JEA, Aasen T, O'Toole EA, Kelsell DP, Zicha D (2005). Connexin interaction patterns in keratinocytes revealed morphologically and by FRET analysis. Journal of Cell Science, 118(7), 1505 - 1514. doi:10.1242/jcs.01733
    • Ong C, Di WL, O'Toole EA, Green A, Callard R, Harper J (2005). LEKTI is required for skin barrier integrity and acts via the PAR-2 signalling pathway.

    2004

    • Ong C, Di WL, Mazereeuw J, Callard R, O'Toole E, Harper J (2004). LEKTI, terminal differentiation of keratinocytes and skin barrier formation.
    • Common JE, Di WL, Davies D, Kelsell DP (2004). Further evidence for heterozygote advantage of GJB2 deafness mutations: a link with cell survival. Journal of Medical Genetics, 41(7), 573 - 575.
    • Gu Y, Di WL, Kelsell DP, Zicha D (2004). Quantitative fluorescence resonance energy transfer (FRET) measurement with acceptor photobleaching and spectral unmixing. Journal of Microscopy, 215(Pt2), 162 - 173.
    • Ong C, Di WL, Mazereeuw J, Callard R, O'Toole E, Harper J (2004). LEKTI, terminal differentiation of keratinocytes and skin barrier formation.
    • Skerrett IM, Di W, Kasperek EM, Kelsell P, Nicholson BJ (2004). Aberrant gating, but a normal expression pattern, underlies the recessive phenotype of the deafness mutant connexin26M34T. The FASEB Journal, 18(7), 860 - 862. doi:10.1096/fj.03-0763fje

    2003

    • Common JE, Di WL, Davies D, Galvin H, Leigh IM, O'Toole EA, Kelsell DP (2003). Cellular mechanisms of mutant connexins in skin disease and hearing loss.. Cell Commun Adhes, 10(4-6), 347 - 351.
    • Kelsell D, Di WL, Monypenny J, Common J, Davies D, Leigh I, Zicha D (2003). Cell death is characteristic of skin disease-associated connexin 31 mutations.
    • Common JEA, Di WL, O'Toole E, Leigh IM, Kelsel DP (2003). Genetic and functional analysis of connexin 26 and 30 in skin disease and deafness.

    2002

    • Di WL, Monypenny J, Common JEA, Kennedy CTC, Holland KA, Rugg EL, Leigh IM, Zicha D, Kelsell DP (2002). Defective trafficking and cell death are characteristic of skin disease-associated connexin 31 mutations in keratinocytes. J INVEST DERMATOL, 119(3), 745 - 745.
    • Common JEA, Becker DL, Di WL, Leigh IM, O'Toole EA, Kelsell DP (2002). Functional studies of human skin disease- and deafness-associated Connexin 30 mutations. Biochemical and Biophysical Research Communications, 298, 651 - 656.
    • Gottfried I, Landau M, Glaser F, Di WL, Ophir J, Mevorah B, Ben-Tal N, Kelsell DPA, K B (2002). A mutation in GJB3 is associated with recessive erythrokeratodermia variabilis (EKV) and leads to defective trafficking of the connexin 31 protein. Human Molecular Genetics, 11(11), 1311 - 1316.
    • Common JEA, Di W, Leigh IM, Kelsell DP (2002). Genetic and functional analysis of connexins in skin disease and deafness..

    2001

    • Kelsell DP, Di W, Common JEA, Rugg EL, Leigh IM (2001). Functional analyses of connexin mutations in keratinocytes. J INVEST DERMATOL, 117(3), 775 - 775.
    • Kelsell DP, Di WL, Houseman MJ (2001). Connexin Mutations in skin disease and hearing Loss. The American Journal of Human Genetics, 68(3), 559 - 568.
    • Houseman MJ, Ellis LA, Pagnamenta A, Di WL, Rickard S, Osborn AH, Dahl HHM, Taylor GR, Bitner-Glindzicz M, Reardon W, Mueller RF, Kelsell DP (2001). Genetic analysis of the connexin-26 M34T variant: identification of genotype M34T/M34T segregating with mild- moderate non-syndromic sensorineural hearing loss. Journal of Medical Genetics, 38(1), 20 - 25.
    • Di WL, Common JE, Kelsell DP (2001). Connexin 26 expression and mutation analysis in epidermal disease. Cell Communication and Adhesion, 8(4-6), 415 - 418.
    • O'Toole EA, Di WL, Common J, Leigh IM, Kelsell DP (2001). Heterogeneity of genetic mutations in erythrokeratodermas.
    • Di W-L, Lachelin GCL, McGarrigle HHG, Thomas NSB, Becker DL (2001). Oestriol and oestradiol increase cell to cell communication and connexin43 protein expression in human myometrium. Molecular Human Reproduction, 7(7), 671 - 679.
    • Di W, Rugg EL, Leigh IM, Kelsell DP (2001). Multiple epidermal connexins are expressed in different keratinocyte subpopulations including connexin 31. Journal of Investigative Dermatology, 117(4), 958 - 964. doi:10.1046/j.0022-202x.2001.01468.x

    1999

    • Di WL, Djahanbakhch O, Kadva A, Street C, Silman R (1999). The pineal and extra-pineal origins of 5-sulphatoxy N-acetyl-serotonin in humans.. J Pineal Res, 26(4), 221 - 226.

    1998

    • Di WL, Kadva A, Djahanbakhch O, Silman R (1998). Radioimmunoassay of bound and free melatonin in plasma.. Clin Chem, 44(2), 304 - 310.
    • Kadva A, Djahanbakhch O, Monson J, Di WL, Silman R (1998). Elevated nocturnal melatonin is a consequence of gonadotropin-releasing hormone deficiency in women with hypothalamic amenorrhea.. J Clin Endocrinol Metab, 83(10), 3653 - 3662. doi:10.1210/jcem.83.10.5155

    1997

    • Di WL, Kadva A, Johnston A, Silman R (1997). Variable bioavailability of oral melatonin.. N Engl J Med, 336(14), 1028 - 1029. doi:10.1056/NEJM199704033361418

    1996

    • Peniston-Bird JF, Di WL, Street CA, Edwards R, Little JA, Silman RE (1996). An enzyme immunoassay for 6-sulphatoxy-melatonin in human urine.. J Pineal Res, 20(2), 51 - 56.
    • Street CA, Di WL, Peniston-Bird JF, Patel S, Sadler P, Silman RE (1996). The purification and characterization of biological 6-sulphatoxymelatonin and comparison with synthetic 6-sulphatoxymelatonin.. J Pineal Res, 20(2), 98 - 114.
    • Kadva A, Di WL, Djahanbakhch O, Monson J, Silman R (1996). Evidence for the Bauman variant in Kallmann's syndrome.. Clin Endocrinol (Oxf), 44(1), 103 - 110.

    1993

    • Peniston-Bird JF, Di WL, Street CA, Kadva A, Stalteri MA, Silman RE (1993). HPLC assay of melatonin in plasma with fluorescence detection.. Clin Chem, 39(11 Pt 1), 2242 - 2247.