Acid sphingomyelinase deficiency does not protect from graft-versus-host disease in transplant recipients with Niemann-Pick disease.

نویسندگان

  • Jakub Tolar
  • Mary Eapen
  • Paul J Orchard
  • Bruce R Blazar
چکیده

performance of the GM-CSF affinity column used to isolate antibodies. If used repeatedly, is it possible that a proportion of high affinity anti–GM-CSF AAs from PAP sera used as a positive control would be retained on the GM-CSF column and released slowly during subsequent purifications of HC IgG? If PAP anti–GMCSF IgG contamination did occur, not only would it account for the immunoblot data given in Figure 1a in their paper,1 where radiolabeled GM-CSF was shown to bind to the bound (IgG) fraction from both HC and PAP IgG, but also for the positive results in bioassays. Evidence of absolute clearance of GM-CSF–bound proteins before column reuse would have been useful in eliminating this possibility. Finally, the functional assays used for GM-CSF, including those used for measuring neutralizing effects, do not appear to have been adequately controlled for specificity. Assays used for measuring GM-CSF activity are not specific as they can respond to a range of cytokines and can be affected by inhibitory components in sera.13 Therefore, if specificity for neutralizing GM-CSF is to be claimed, then it is necessary to show that the affinity-purified antibodies do not neutralize other cytokines, such as interleukin-3, which can be tested using the TF1 cell-proliferation assay.13

برای دانلود رایگان متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

منابع مشابه

[Niemann-Pick disease types A and B].

The molecular basis of Niemann-Pick disease, type A and B, has been confirmed by detection of mutations causing deficiency of the acid sphingomyelinase activity in the patients. It has been shown that mutations, which cause no activity of acid sphingomyelinase, are responsible for the type A and mutations which cause residual activities of the enzyme are responsible for the type B. Acid sphingo...

متن کامل

بررسی یک مورد بیماری Niemann Pick تیپ A

Niemann Pick type A is a very rare hereditary disease with an incidence 1 in 20000-40000 live birth, which is calassified as a shingolipidoses. The disease is marked by the abnormal accumulation of sphingomyelin in most tissues, secondary to sphingomylinase deficiency. The most clinical manifestations are: Splenohepatomegaly–cherry red maculae-neuropathologic findings . This is a ...

متن کامل

Recombinant CD40L treatment protects allogeneic murine bone marrow transplant recipients from death caused by herpes simplex virus-1 infection.

Posttransplant infection associated with host immune deficiency is the major cause of nonrelapse mortality of human bone marrow transplant recipients. In a new murine model of posttransplant infection, allogeneic bone marrow transplant recipients were infected with herpes simplex virus-1 (HSV-1) via intraperitoneal inoculation 12 weeks after transplantation. Allogeneic transplant recipients wit...

متن کامل

A 16 Month Survey of Cyclosporine Utilization Evaluation in Allogeneic Hematopoietic Stem Cell Transplant Recipients

Objectives: Graft versus host disease (GVHD) is a life threatening reaction in the stem cell transplantation process. Nowadays Cyclosporine is the most commonly utilized agent for GVHD prophylaxis and it has a major role in successful transplantation. Cyclosporine has been applied for many years in this field but it could be stated that currently no general consensus is available for its optima...

متن کامل

Anesthetic Management in a Child With Niemann-Pick Disease

Niemann-Pick is a lipid storage disease that results from a lysosomal enzyme deficiency (sphingomyelinase). It has different presentations, and it may affect various organs such as the central nervous system, kidney, liver, and spleen. Due to the complexity of the disease, careful perianesthetic management is necessary in order to reduce the risks and sequels. As there is little evidence availa...

متن کامل

ذخیره در منابع من


  با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید

عنوان ژورنال:
  • Blood

دوره 115 2  شماره 

صفحات  -

تاریخ انتشار 2010