Adenosine deaminase deficiency in adults. (1997)
Ozsahin, H, Arredondo-Vega, F X, Santisteban, I, Fuhrer, H, Tuchschmid, P, Jochum, W, ...
Adenosine deaminase (ADA) deficiency typically causes severe combined immunodeficiency (SCID) in infants. We report metabolic, immunologic, and genetic findings in two ADA-deficient adults with...
Hershfield, M S, Chaffee, S, Koro-Johnson, L, Mary, A, Smith, A A, Short, S A
Modification by covalent attachment of polyethylene glycol (PEG) can reduce the immunogenicity and prolong the circulating life of proteins, but the utility of this approach for any protein is...
Begley, C G, Aplan, P D, Davey, M P, Nakahara, K, Tchorz, K, Kurtzberg, J, ...
We have studied a leukemic stem-cell line, DU.528, that is able to differentiate into myeloid and lymphoid cells. The leukemic cells have a translocation between chromosomes 1 and 14,...
Weinberg, K, Hershfield, M S, Bastian, J, Kohn, D, Sender, L, Parkman, R, ...
Adenosine deaminase (ADA) deficiency causes severe combined immune deficiency (SCID) by interfering with the metabolism of deoxyadenosine, which is toxic to T lymphocytes at all stages of...
Arredondo-Vega, F X, Kurtzberg, J, Chaffee, S, Santisteban, I, Reisner, E, Povey, M S, ...
T lymphocytes cultured from a patient (T.D.) with adenosine deaminase (ADA) deficiency expressed ADA activity in the normal range, inconsistent with her severe immunodeficiency, metabolic...
Involvement of the TCL5 gene on human chromosome 1 in T-cell leukemia and melanoma.
Finger, L R, Kagan, J, Christopher, G, Kurtzberg, J, Hershfield, M S, Nowell, P C, ...
We analyzed a t(1;14)(p32;q11) chromosomal translocation in a human lymphohemopoietic stem cell line derived from a patient with acute T-lymphoblastic leukemia. The chromosomal joining on the 1p+...
Hershfield, M S, Kurtzberg, J, Harden, E, Moore, J O, Whang-Peng, J, Haynes, B F
Selective failure of lymphoid development occurs in genetic deficiency of adenosine deaminase (ADA). We examined the in vivo effects of a potent inhibitor of ADA, 2'-deoxycoformycin, which was used...
Accumulation of dATP derived from 2'-deoxyadenosine (dAdo), causing inhibition of ribonucleotide reductase and depletion of the other deoxynucleotide substrates required for DNA synthesis, has been...
We have examined the basis for the recently reported, but unexplained deficiency of S-adenosylhomocysteine hydrolase (AdoHcyase) in the erythrocytes of patients with genetic deficiencies of purine...
Hershfield, M S, Kredich, N M, Ownby, D R, Ownby, H, Buckley, R
The cytotoxic nucleoside 2'-deoxyadenosine is excreted in excessive amounts by individuals with genetic deficiency of adenosine deaminase, and may be in part responsible for the severe combined...
Davey, M P, Bongiovanni, K F, Kaulfersch, W, Quertermous, T, Seidman, J G, Hershfield, M S, ...
The use of probes to genes (IG and TCRB) encoding immunoglobulins (IG) and the beta chain of the T-cell antigen receptor (TCRB), respectively, have become a sensitive means to assess clonality and...
The mouse lethal nonagouti (a(x)) mutation deletes the S-adenosylhomocysteine hydrolase (Ahcy) gene.
Miller, M W, Duhl, D M, Winkes, B M, Arredondo-Vega, F, Saxon, P J, Wolff, G L, ...
The lethal nonagouti (a(x)) mutation is a hypomorphic allele of the agouti coat color locus which, when homozygous, also leads to embryonic death around the time of implantation. To understand the...
Chaffee, S, Mary, A, Stiehm, E R, Girault, D, Fischer, A, Hershfield, M S
Polyethylene glycol (PEG)-modified bovine adenosine deaminase (ADA) is used for replacement therapy of severe combined immunodeficiency disease due to inherited ADA deficiency. We monitored IgG...
S-Adenosylhomocysteine hydrolase from human placenta. Affinity purification and characterization.
Hershfield, M S, Aiyar, V N, Premakumar, R, Small, W C
S-Adenosylhomocysteine hydrolase (EC 3.3.1.1) was purified to homogeneity from human placenta by using S-adenosylhomocysteine-agarose affinity chromatography. The enzyme is a tetramer with a native...
S-Adenosylhomocysteine hydrolase (AdoHcyase) has previously been identified as a cytoplasmic adenosine and cyclic AMP binding protein. In order to examine the relationship between the adenosine and...
Arredondo-Vega, F X, Santisteban, I, Daniels, S, Toutain, S, Hershfield, M S
Adenosine deaminase (ADA) deficiency causes lymphopenia and immunodeficiency due to toxic effects of its substrates. Most patients are infants with severe combined immunodeficiency disease (SCID),...
Hershfield, M S, Chaffee, S, Koro-Johnson, L, Mary, A, Smith, A A, Short, S A
Modification by covalent attachment of polyethylene glycol (PEG) can reduce the immunogenicity and prolong the circulating life of proteins, but the utility of this approach for any protein is...
Begley, C G, Aplan, P D, Davey, M P, Nakahara, K, Tchorz, K, Kurtzberg, J, ...
We have studied a leukemic stem-cell line, DU.528, that is able to differentiate into myeloid and lymphoid cells. The leukemic cells have a translocation between chromosomes 1 and 14,...
Weinberg, K, Hershfield, M S, Bastian, J, Kohn, D, Sender, L, Parkman, R, ...
Adenosine deaminase (ADA) deficiency causes severe combined immune deficiency (SCID) by interfering with the metabolism of deoxyadenosine, which is toxic to T lymphocytes at all stages of...
Arredondo-Vega, F X, Kurtzberg, J, Chaffee, S, Santisteban, I, Reisner, E, Povey, M S, ...
T lymphocytes cultured from a patient (T.D.) with adenosine deaminase (ADA) deficiency expressed ADA activity in the normal range, inconsistent with her severe immunodeficiency, metabolic...
Involvement of the TCL5 gene on human chromosome 1 in T-cell leukemia and melanoma.
Finger, L R, Kagan, J, Christopher, G, Kurtzberg, J, Hershfield, M S, Nowell, P C, ...
We analyzed a t(1;14)(p32;q11) chromosomal translocation in a human lymphohemopoietic stem cell line derived from a patient with acute T-lymphoblastic leukemia. The chromosomal joining on the 1p+...
Hershfield, M S, Kurtzberg, J, Harden, E, Moore, J O, Whang-Peng, J, Haynes, B F
Selective failure of lymphoid development occurs in genetic deficiency of adenosine deaminase (ADA). We examined the in vivo effects of a potent inhibitor of ADA, 2'-deoxycoformycin, which was used...
Accumulation of dATP derived from 2'-deoxyadenosine (dAdo), causing inhibition of ribonucleotide reductase and depletion of the other deoxynucleotide substrates required for DNA synthesis, has been...
We have examined the basis for the recently reported, but unexplained deficiency of S-adenosylhomocysteine hydrolase (AdoHcyase) in the erythrocytes of patients with genetic deficiencies of purine...
Hershfield, M S, Kredich, N M, Ownby, D R, Ownby, H, Buckley, R
The cytotoxic nucleoside 2'-deoxyadenosine is excreted in excessive amounts by individuals with genetic deficiency of adenosine deaminase, and may be in part responsible for the severe combined...
Davey, M P, Bongiovanni, K F, Kaulfersch, W, Quertermous, T, Seidman, J G, Hershfield, M S, ...
The use of probes to genes (IG and TCRB) encoding immunoglobulins (IG) and the beta chain of the T-cell antigen receptor (TCRB), respectively, have become a sensitive means to assess clonality and...
The mouse lethal nonagouti (a(x)) mutation deletes the S-adenosylhomocysteine hydrolase (Ahcy) gene.
Miller, M W, Duhl, D M, Winkes, B M, Arredondo-Vega, F, Saxon, P J, Wolff, G L, ...
The lethal nonagouti (a(x)) mutation is a hypomorphic allele of the agouti coat color locus which, when homozygous, also leads to embryonic death around the time of implantation. To understand the...
Chaffee, S, Mary, A, Stiehm, E R, Girault, D, Fischer, A, Hershfield, M S
Polyethylene glycol (PEG)-modified bovine adenosine deaminase (ADA) is used for replacement therapy of severe combined immunodeficiency disease due to inherited ADA deficiency. We monitored IgG...
S-Adenosylhomocysteine hydrolase from human placenta. Affinity purification and characterization.
Hershfield, M S, Aiyar, V N, Premakumar, R, Small, W C
S-Adenosylhomocysteine hydrolase (EC 3.3.1.1) was purified to homogeneity from human placenta by using S-adenosylhomocysteine-agarose affinity chromatography. The enzyme is a tetramer with a native...
S-Adenosylhomocysteine hydrolase (AdoHcyase) has previously been identified as a cytoplasmic adenosine and cyclic AMP binding protein. In order to examine the relationship between the adenosine and...
Arredondo-Vega, F X, Santisteban, I, Daniels, S, Toutain, S, Hershfield, M S
Adenosine deaminase (ADA) deficiency causes lymphopenia and immunodeficiency due to toxic effects of its substrates. Most patients are infants with severe combined immunodeficiency disease (SCID),...
Arredondo-Vega, F. X., Santisteban, I., Kelly, S., Schlossman, C. M., Umetsu, D. T., Hershfield, M. S.
Adenosine deaminase (ADA) deficiency usually causes severe combined immune deficiency in infancy. Milder phenotypes, with delayed or late onset and gradual decline in immune function, also occur and...