Specification of the phenotype required for men with monoamine oxidase type A deficiency

  title={Specification of the phenotype required for men with monoamine oxidase type A deficiency},
  author={Johannes Hebebrand and Birgit Klug},
  journal={Human Genetics},
Brunner et al. (1993 a, b) reported on a Dutch family, in which complete monoamine oxidase type A (MAO-A) deficiency due to a point mutation in the respective gene is associated with a "recognizable behavioral phenotype" (all quotations refer to Brunner et al. 1993 a, b) that includes distrubed regulation of impulsive aggression. Understandably, these findings received considerable coverage in both the scientific (Morell 1993) and lay press, because for the first time aggressive behavior had… 

Novel monoamine oxidase A knock out mice with human-like spontaneous mutation

Findings consolidate evidence linking MAO A to aggression and highlight subtle yet distinctive phenotypical characteristics in a novel line of mutant mice harboring a spontaneous point nonsense mutation.

Monoamine oxidase A and A/B knockout mice display autistic-like features.

It is suggested that the neurochemical imbalances induced by MAOA deficiency (either by itself or in conjunction with lack of MAOB) may result in an array of abnormalities similar to those observed in ASDs.

Behavioral outcomes of monoamine oxidase deficiency: preclinical and clinical evidence.

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In a recent article in this journal, Sulisalo et al. (1995) addressed the problem of potential linkage (i.e. locus) heterogeneity of autosomal recessive cartilage-hair hypoplasia by analysing pedigrees from six different populations and found that their power to disclose linkage heterogeneity was limited.

New insights into Brunner syndrome and potential for targeted therapy

Brunner syndrome should be considered as a cause of mild ID with paroxysmal behavioural symptoms and Cautious treatment with a serotonin reuptake inhibitor, dietary modifications and avoidance of medications contraindicated in patients on monoamine oxidase inhibitors can improve symptoms.

XXVI-7 Neurogenetics of Personality Disorders

The behavioural predisposition of an individual is commonly referred to as temperament or personality. In genetics, personality is generally defined as the characteristic manner and style of an

MAOA Gene Associated with Aggressive Behavior in Humans

The inheritance pattern and clinically significant polymorphisms of MAOA gene has been described and the relationship between metabolomic, genetic makeup which is surrounding with the environmental factors is understood.

Child and adolescent psychiatric genetics

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Monoamine Oxidase-A in Borderline Personality Disorder and Antisocial Personality Disorder

Monoamine oxidase A (MAO-A) is a brain enzyme that serves several physiologic functions, including metabolism of monoamine neurotransmitters and induction of pro-apoptotic signaling pathways.



Abnormal behavior associated with a point mutation in the structural gene for monoamine oxidase A.

Analytical results indicate that isolated complete MAOA deficiency in this family is associated with a recognizable behavioral phenotype that includes disturbed regulation of impulsive aggression.

X-linked borderline mental retardation with prominent behavioral disturbance: phenotype, genetic localization, and evidence for disturbed monoamine metabolism.

Normal platelet MAOB activity suggests that the unusual behavior pattern in this family may be caused by isolated MAOA deficiency, which is compatible with a primary defect in the structural gene for MAOA and/or monoamine oxidase type B (MAOB).

Evidence found for a possible 'aggression gene'.

Evidence is reported suggesting that a mutation in the gene for monoamine oxidase A (MAOA) may underlie the aggressive and sometimes violent behavior displayed by certain males in this family.

A critical analysis of data presented in eight studies favouring X-linkage of bipolar illness with special emphasis on formal genetic aspects

Sex-dependently analyzing segregation ratios and clinical data presented in eight positive X-linkage studies shows that affected males have significantly fewer offspring, a lower mean age and a higher bipolar to unipolar ratio than affected females.

A Critical Appraisal of X-Linked Bipolar Illness

An evaluation of the segregation patterns in the pedigrees that have been analysed for X-linkage over the past 20 years does not support the assumption that a subgroup of manic-depressive illness follows an X-linked dominant mode of inheritance.

A critical evaluation of clinical data presented in eight positive X - linkage studies of bipolar illness

  • Hum Genet
  • 1992

a) all of the individuals that we consider unaffected, had at least completed normal primary education. In contrast, only one out of eight affected males has completed normal primary education

  • 1993

1993 a, b). In their reply Brunner and Ropers now point out that all predigree members whose DNA

  • 1993