Matrix-Valued Little q-Jacobi Polynomials

Matrix-valued analogues of the little q-Jacobi polynomials are introduced and studied. For the 2x2-matrix-valued little q-Jacobi polynomials explicit expressions for the orthogonality relations, Rodrigues formula, three-term recurrence relation and their relation to matrix-valued q-hypergeometric series and the scalar-valued little q-Jacobi polynomials are presented. The study is based on a matrix-valued q-difference operator, which is a q-analogue of Tirao's matrix-valued hypergeometric differential operator.Comment: 16 pages, various corrections and minor additions, incorporating referee's comment

Neonatal umbilical cord blood transplantation halts skeletal disease progression in the murine model of MPS-I

Umbilical cord blood (UCB) is a promising source of stem cells to use in early haematopoietic stem cell transplantation (HSCT) approaches for several genetic diseases that can be diagnosed at birth. Mucopolysaccharidosis type I (MPS-I) is a progressive multi-system disorder caused by deficiency of lysosomal enzyme α-L-iduronidase, and patients treated with allogeneic HSCT at the onset have improved outcome, suggesting to administer such therapy as early as possible. Given that the best characterized MPS-I murine model is an immunocompetent mouse, we here developed a transplantation system based on murine UCB. With the final aim of testing the therapeutic efficacy of UCB in MPS-I mice transplanted at birth, we first defined the features of murine UCB cells and demonstrated that they are capable of multi-lineage haematopoietic repopulation of myeloablated adult mice similarly to bone marrow cells. We then assessed the effectiveness of murine UCB cells transplantation in busulfan-conditioned newborn MPS-I mice. Twenty weeks after treatment, iduronidase activity was increased in visceral organs of MPS-I animals, glycosaminoglycans storage was reduced, and skeletal phenotype was ameliorated. This study explores a potential therapy for MPS-I at a very early stage in life and represents a novel model to test UCB-based transplantation approaches for various diseases

Dried Blood Spot Analysis: An Easy And Reliable Tool To Monitor The Biochemical Effect Of Hematopoietic Stem Cell Transplantation In Hurler Syndrome Patients In A Multi-Center International Setting

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