Kappa-chain deficiency.

نویسندگان

  • G M Bernier
  • J R Gunderman
  • F B Ruymann
چکیده

A decreased concentration of immunogesting that the synthesis of molecules globulin molecules of one light chain bearing K-chains was decreased. The type (K) was found in a young girl with patient improved considerably with the recurrent respiratory infections and regular administration of 7-globulin. It diarrhea. She had, in addition, deis possible that biological as well as creased yA and yE globulin, partial chemical differences exist between imalbinism, and intestinal lactase demunoglobulins of the two light-chain ficiency. No eosinophils were detected types and that an optimal immune In blood, bone marrow, or nasal response is dependent on a normal smears. Radiolabeled Kand Atype complement of Kand A-chains. molecules survived equally well, sugI NCREASED SUSCEPTIBILITY to pyogenic infections, has been a common manifestation of immunoglobulin deficiency. In patients with frequent infections, the degree of immunoglobulin deficiency has been variable in that some patients exhibit extreme depression of all immunoglobulin classes and others are deficient in only a single class.’3 In some patients with recurrent infections, decreased concentration of even one or two subclasses of 1G globulin has been implicated.4 Class and subclass distinctions of immunoglobulins are based on differences in heavy chains, and immune disorders attendant on deficiencies of a class or subclass have been taken as presumptive evidence for the biological importance of the deficient class or subclass. Immunoglobulins also differ by virtue of their light chains (K and A), and selective deficiency of one or the other light chain might be expected to result in compromised humoral immunity. However, to date, deficiency of immunoglobulins of a single light-chain type has not been described. This report concerns a young girl with repeated respiratory infections and diarrhea whose serum and secretory immunoglobulins were found to have a selective deficiency of K-type molecules. The survival of administered 7-globulin was normal with respect to both light-chain types, suggesting that K-chain synthesis was selectively suppressed.

برای دانلود 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

Expression of an idiotype (Id-460) during in vivo anti-dinitrophenyl antibody responses. I. Mapping of genes for Id-460 expression to the variable region of immunoglobulin heavy-chain locus and to the variable region of immunoglobulin kappa-light-chain locus

The genetic contro of the expression of an idiotype (Id-460) associated with the 2,4-dinitrophenyl (DNP)-binding BALB/c myeloma protein MOPC 460 was studied using congenic strains of mice. It was shown that the expression of high levels of Id-460 during secondary in vivo anti-DNP-ovalbumin responses was determined by genes governing immunoglobulin heavy-chain variable and kappa-light chain vari...

متن کامل

Development and characterization of polyclonal antibody against human kappa light chain in rabbit

Polyclonal antibodies against kappa light chain are used to diagnose diseases producing free light chain. The kappa and lambda light chains are products of immunoglobulin synthesis and released into the circulation in minor amounts such as serum, cerebrospinal fluid, urine and synovial fluid in normal condition. The purpose of this study was the production and purification of polyclonal immunog...

متن کامل

Burkitt's lymphoma in AIDS: cytogenetic study.

Cytogenetic studies were done in two cases of Burkitt's lymphoma in homosexual individuals with possible acquired immune deficiency syndrome (AIDS). The chromosomal abnormalities found are consistent with those previously described in the nonendemic form of Burkitt's lymphoma, with one of the two patients having the variant translocation, t(8;22). The production of the kappa light chain immunog...

متن کامل

deficiency: implications for diagnosis and therapy

Objectives-Carnitine palmitoyltransferase (CPT) deficiency is one of the most common defects of mitochondrial fatty acid oxidation. Two different enzymes (CPT-I and CPT-II) are involved. Due to problems in measuring enzyme activity, relatively little is known about the substrate specificity of each of the human enzymes. This is of considerable importance in the treatment of patients. The object...

متن کامل

Tissue-Specific Strategies of the Very-Long Chain Acyl-CoA Dehydrogenase-Deficient (VLCAD−/−) Mouse to Compensate a Defective Fatty Acid β-Oxidation

Very long-chain acyl-CoA dehydrogenase (VLCAD)-deficiency is the most common long-chain fatty acid oxidation disorder presenting with heterogeneous phenotypes. Similar to many patients with VLCADD, VLCAD-deficient mice (VLCAD(-/-)) remain asymptomatic over a long period of time. In order to identify the involved compensatory mechanisms, wild-type and VLCAD(-/-) mice were fed one year either wit...

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

برای دانلود 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

عنوان ژورنال:
  • Blood

دوره 40 6  شماره 

صفحات  -

تاریخ انتشار 1972