Urea cycle disorder

Urea Cycle Disorder

Urea cycle disorder (== Template:IPA ==

The Template:IPA (International Phonetic Alphabet) is a system of phonetic notation based primarily on the Latin alphabet. It was devised by the International Phonetic Association in the late 19th century as a standardized representation of the sounds of spoken language.

Pronunciation

The pronunciation of the term "IPA" is /aɪ piː eɪ/ in English.

Etymology

The term "IPA" is an acronym for the International Phonetic Alphabet. The International Phonetic Association, founded in 1886, created the IPA to provide a single, universal system for the transcription of spoken language.

Related Terms

Phonetic notation: A system used to visually represent the sounds of speech. The IPA is one type of phonetic notation.
Phonetics: The study of the physical sounds of human speech. It is concerned with the physical properties of speech sounds (phonemes), and the processes of their physiological production, auditory reception, and neurophysiological perception.
Phonology: The study of the way sounds function within a particular language or languages. While phonetics concerns the physical production, acoustic transmission and perception of the sounds of speech, phonology describes the way sounds function within a particular language or languages.
Transcription (linguistics): The systematic representation of spoken language in written form. The source of the words transcribe and transcription, the term means "to write across" in Latin, and it's the process of converting spoken language into written form. In linguistics, this is often done using the IPA.

External links

Medical encyclopedia article on Urea cycle disorder
Wikipedia's article - Urea cycle disorder

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Languages: - East Asian 中文, 日本, 한국어, South Asian हिन्दी, Urdu, বাংলা, తెలుగు, தமிழ், ಕನ್ನಡ,
Southeast Asian Indonesian, Vietnamese, Thai, မြန်မာဘာသာ, European español, Deutsch, français, русский, português do Brasil, Italian, polski) is a genetic disorder caused by a mutation that results in a deficiency of one of the six enzymes in the urea cycle. These enzymes are responsible for removing ammonia from the blood stream. The urea cycle involves a series of biochemical steps in which nitrogen, a waste product of protein metabolism, is removed from the blood and converted to a compound called urea in the blood. Normally, the urea is transferred into the urine and removed from the body. In urea cycle disorders, the nitrogen accumulates in the form of ammonia, a highly toxic substance, resulting in hyperammonemia (elevated blood ammonia). Ammonia then reaches the brain through the blood, where it can cause irreversible brain damage, coma and/or death.

The urea cycle consists of six consecutive reactions that occur in the mitochondria and cytosol of the liver. The cycle is regulated by N-acetylglutamate, which ensures that the rate of urea production is adequate to the body's needs.

The six enzymes involved in the urea cycle are:

The term "urea cycle disorder" is derived from the Latin urea, meaning "urine", and the Greek kyklos, meaning "circle".

Related terms include:

Urea cycle disorder