scholarly journals II. Adrenal Cortex and Steroid 21-Hydroxylase Autoantibodies in Children with Organ-Specific Autoimmune Diseases: Markers of High Progression to Clinical Addison’s Disease

1997 ◽  
Vol 82 (3) ◽  
pp. 939-942
Author(s):  
Corrado Betterle ◽  
Marina Volpato ◽  
Bernard Rees Smith ◽  
Jadwiga Furmaniak ◽  
Shu Chen ◽  
...  
Keyword(s):  
Autoimmune Diseases ◽  
Adrenal Cortex ◽  
Addison’S Disease ◽  
Adrenal Function ◽  
Side Chain ◽  
Addison's Disease ◽  
Side Chain Cleavage ◽  
Chain Cleavage ◽  
Organ Specific ◽  
Cleavage Enzyme

Abstract Adrenal cortex autoantibodies (ACA) were measured by immunofluorescence in 808 children with organ-specific autoimmune diseases without adrenal insufficiency. ACA were found in 14 children (1.7%), mostly in hypoparathyroidism (48%). Ten ACA-positive and 12 ACA-negative children were followed up for a maximum of 10 yr by evaluation of adrenocortical function (ACTH test) and autoantibody status. In all patients steroid-producing cell autoantibodies were assessed by immunofluorescence and autoantibodies to steroid 21-hydroxylase, 17α-hydroxylase, and cytochrome P450 side-chain cleavage enzyme by immunoprecipitation assay. All 10 ACA-positive patients were positive for 21-hydroxylase autoantibodies. Six were positive for steroid-producing cell autoantibodies and 5 also for autoantibodies to 17α-hydroxylase and/or P450 side-chain cleavage enzyme. Overt Addison’s disease developed in 9 (90%) ACA/21-OH-antibody-positive children after 3–121 months, and 1 remaining child had subclinical hypoadrenalism. By contrast, all ACA/21-OH antibody-negative children maintained normal adrenal function. Adrenal failure was not related to ACA titres, sex, adrenal function, type of preexisting autoimmune disorder, or human leucocyte antigens D-related status. In conclusion, in children with autoimmune endocrine diseases, ACA/21-hydroxylase autoantibodies are important predictive markers for the development of Addison’s disease.

scholarly journals I. Adrenal Cortex and Steroid 21-Hydroxylase Autoantibodies in Adult Patients with Organ-Specific Autoimmune Diseases: Markers of Low Progression to Clinical Addison’s Disease

1997 ◽  
Vol 82 (3) ◽  
pp. 932-938 ◽  
Author(s):  
Corrado Betterle ◽  
Marina Volpato ◽  
Bernard Rees Smith ◽  
Jadwiga Furmaniak ◽  
Shu Chen ◽  
...  
Keyword(s):  
Autoimmune Diseases ◽  
Prospective Study ◽  
Adrenal Cortex ◽  
Addison’S Disease ◽  
Adrenal Function ◽  
Adult Patients ◽  
Addison's Disease ◽  
Chain Cleavage ◽  
Organ Specific ◽  
A Prospective Study

Abstract Adrenal cortex antibodies (ACA) were measured by immunofluorescence in 8840 adult patients with organ-specific autoimmune diseases without overt hypoadrenalism. Sixty-seven (0.8%) patients were ACA-positive, with the highest prevalence in those with premature ovarian failure (8.9%). Forty-eight ACA-positive and 20 ACA-negative individuals were enrolled into a prospective study. Antibodies to steroid 21-hydroxylase (21-OH), steroid 17α-hydroxylase (17α-OH) and cytochrome P450 side chain cleavage enzyme (P450scc) were measured by immunoprecipitation assay. Human leucocyte antigens D-related (HLA-DR) genotyping was also carried out and adrenal function assessed by ACTH test. On enrollment, 75% of ACA-positive patients had a normal adrenal function, while 25% revealed a subclinical hypoadrenalism. 21-OH antibodies were positive in 91% of ACA-positive sera. Eleven patients were positive for steroid-cell antibodies by immunofluorescence, and 9 revealed a positivity for antibodies to 17α-OH and/or P450scc. During the prospective study, overt Addison’s disease developed in 21% and subclinical hypoadrenalism in 29% of ACA-positive patients, while 50% maintained normal adrenal function. Progression to Addison’s disease was more frequent in patients with subclinical hypoadrenalism, high titers of ACA and higher levels of 21-OH antibodies, complement-fixing ACA and HLA-DR3 status. All 20 persistently ACA-negative patients were also negative for antibodies to 21-OH, 17α-OH, and P450scc, and all maintained normal adrenal function during follow-up. In conclusion, the detection of ACA/21-OH antibodies in adults is a marker of low progression toward clinical Addison’s disease. .

P450 side-chain cleavage enzyme autoantibodies in canine Addison's disease

2013 ◽  
pp. 1-1
Author(s):  
Alisdair Boag ◽  
Kerry McLaughlin ◽  
Mike Christie ◽  
Peter Graham ◽  
Harriet Syme ◽  
...  
Keyword(s):  
Addison’S Disease ◽  
Side Chain ◽  
Addison's Disease ◽  
Side Chain Cleavage ◽  
Chain Cleavage ◽  
Cleavage Enzyme

scholarly journals Autoantibodies against 21-hydroxylase and side-chain cleavage enzyme in autoimmune Addison's disease are mainly immunoglobulin G1

2004 ◽  
pp. 49-56 ◽  
Author(s):  
AS Boe ◽  
G Bredholt ◽  
PM Knappskog ◽  
TO Hjelmervik ◽  
G Mellgren ◽  
...  
Keyword(s):  
Expression System ◽  
Addison’S Disease ◽  
Side Chain ◽  
Addison's Disease ◽  
Tissue Destruction ◽  
Affinity Tag ◽  
Western Blots ◽  
Side Chain Cleavage ◽  
Chain Cleavage ◽  
Cleavage Enzyme

OBJECTIVE: Immunoglobulin G (IgG) antibodies to the steroidogenic enzymes 21-hydroxylase (21OH) and side-chain cleavage enzyme (SCC) are important diagnostic markers for autoimmune Addison's disease and autoimmune polyendocrine syndromes (APS) types I and II. The characterization of autoantibody (IgG) subclasses may reveal information on how tIssue destruction takes place; therefore, IgG subtypes of anti-21OH and anti-SCC antibodies from sera of patients with Addison's disease, APS I and APS II were determined using recombinant 21OH and SCC. METHODS: SCC(51-521) and his-SCC(51-521) were expressed by pET-scc in the Escherichia coli strain BL21 Star (DE3) and inclusion bodies were purified. Full-length, human 21OH fused to an N-terminal 6x histidine affinity tag was expressed in insect cells by using the baculovirus expression system bac-to-bac. Western blots were used to investigate the IgG subtype(s) of the autoantibodies against 21OH and SCC in patients and healthy blood donors. RESULTS: All anti-SCC positive sera (n=10) contained autoantibodies of the IgG1 subclass, while four out of ten also contained IgG3. All anti-21OH positive sera (n=16) had autoantibodies exclusively against IgG1. Sera from 20 healthy subjects did not show any reactivity against 21OH or SCC. CONCLUSIONS: The finding of a predominating IgG1 response against 21OH and SCC may suggest that T helper (Th) cells of the Th1 subclass are involved in destruction of the adrenal cortex in patients with autoimmune Addison's disease.

scholarly journals Autoantibodies against Cytochrome P450 Side-Chain Cleavage Enzyme in Dogs (Canis lupus familiaris) Affected with Hypoadrenocorticism (Addison’s Disease)

PLoS ONE ◽  
2015 ◽  
Vol 10 (11) ◽  
pp. e0143458 ◽  
Author(s):  
Alisdair M. Boag ◽  
Michael R. Christie ◽  
Kerry A. McLaughlin ◽  
Harriet M. Syme ◽  
Peter Graham ◽  
...  
Keyword(s):  
Cytochrome P450 ◽  
Canis Lupus ◽  
Addison’S Disease ◽  
Side Chain ◽  
Addison's Disease ◽  
Canis Lupus Familiaris ◽  
Side Chain Cleavage ◽  
Chain Cleavage ◽  
Cleavage Enzyme

scholarly journals Autoantibodies to steroidogenic enzymes in patients with premature ovarian failure with and without Addison's disease

2003 ◽  
pp. 565-570 ◽  
Author(s):  
C Dal Pra ◽  
S Chen ◽  
J Furmaniak ◽  
BR Smith ◽  
B Pedini ◽  
...  
Keyword(s):  
Autoimmune Diseases ◽  
Premature Ovarian Failure ◽  
Addison’S Disease ◽  
Ovarian Failure ◽  
Lower Proportion ◽  
Side Chain ◽  
Addison's Disease ◽  
Steroidogenic Enzymes ◽  
Chain Cleavage ◽  
Patients At Risk

DESIGN: Adrenal cortex autoantibodies (ACA), steroid-producing cell autoantibodies (StCA) and autoantibodies (Abs) to steroidogenic enzymes in three groups of patients with premature ovarian failure (POF), 15 with autoimmune Addison's disease (AD), 26 with non-adrenal autoimmune diseases and 31 with isolated POF, have been assessed. METHODS: ACA and StCA were measured using an immunofluorescence technique. Abs to 21-hydroxylase (21-OH), to 17alpha-hydroxylase (17alpha-OH) and to cytochrome P450 side-chain cleavage (P450scc) were measured using an immunoprecipitation assay. RESULTS: Seventy-three percent of patients with POF and AD were positive for StCA, 93% for 17alpha-OH and/or P450scc Abs, 93% for ACA and 100% for 21-OH Abs. Among patients with POF and non-adrenal autoimmune diseases, 8% were positive for StCA, 12% for 17alpha-OH and/or P450scc Abs, and 8% and 12% for ACA and 21-OH Abs respectively. StCA, 17alpha-OH and/or P450scc Abs were all found in 10% of patients with isolated POF, and 13% had ACA and 21-OH Abs. All StCA-, 17alpha-OH- and/or P450scc Abs-positive patients were also positive for ACA and 21-OH Abs. Two patients with isolated POF who were ACA and 21-OH Ab positive developed AD 3 and 5 Years after the onset of POF. CONCLUSION: This study has shown that, when POF is associated with AD, StCA, 17alpha-OH and/or P450scc Abs are present in the majority of patients, while in the other two groups these Abs are detectable in a much lower proportion of patients. Measurement of ACA/21-OH Abs in some patients with POF may be important in identifying patients at risk of developing overt AD.

Specificity of the cholesterol side-chain cleavage enzyme system of adrenal cortex

Steroids ◽  
1977 ◽  
Vol 30 (4) ◽  
pp. 439-453 ◽  
Author(s):  
Shlomo Burstein ◽  
R.C. Nickolson ◽  
Chang Y. Byon ◽  
Howard L. Kimball ◽  
Marcel Gut
Keyword(s):  
Adrenal Cortex ◽  
Enzyme System ◽  
Side Chain ◽  
Side Chain Cleavage ◽  
Chain Cleavage ◽  
Cleavage Enzyme
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