HAL deficiency i.e histidine ammonia-lyase (HAL) deficiency or histidase deficiency, hyperhistidinemia or Histidinemia is a rare, autosomal recessive hereditary metabolic disorder.
- It is caused by a deficiency of the enzyme histidase, which is necessary for metabolizing the amino acid histidine. As a result, elevated levels of histidine are present in the blood.
- Along with histidine, there are excessive amounts of imidazole pyruvic acid and other imidazole metabolism products that are excreted in the urine.
Histidine is an essential amino acid that is not formed in the body. It is required for,
- Producing white and red blood cells, producing ferritin and hemoglobin.
- Reducing inflammation and oxidative stress
- Helping regulate feeding behavior and energy metabolism
- Regulating levels of iron, copper, molybdenum, zinc and manganese
- Producing histamine, a neurotransmitter.
- Producing carnosine- leads to the reduction of plaque buildup in the arteries.
- Repairing tissue and wounds.
- Regulating the pH value of the blood.
- Protecting the skin by producing urocanic acid, an absorber of UV light.
Rheumatoid arthritis,Anaemia,Psychological disorders such as anxiety and schizophrenia,Deafness,Colds and Allergies,Poor growth in infants,Heart and kidney ailments
You can get the histidine that your body needs by eating a well-balanced, healthy diet that includes enough protein foods like meat, fish, poultry, eggs, legumes, nuts and seeds. Adults need an amount of 8 to 12 milligrams of histidine per kilogram of body weight, daily. Regulating the amount of histidine-rich food brings the histidine level back to normal.
Behavioural abnormality,Hyperactivity,Psychomotor retardation,Affected skills for learning like reading or writing, coordination, self-control or attention that interfere with the ability to learn