33 下列那一個胺基酸可以代謝成α-ketoglutarate，然後進入citric acid cycle？

 Glycine Proline Alanine Tyrosine

no answer: the answer should be glutamate

Krebs cycle

α-Ketoglutarate is a key intermediate in the Krebs cycle, coming after isocitrate and before succinyl CoA. Anaplerotic reactions can replenish the cycle at this juncture by synthesizing α-ketoglutarate from transamination of glutamate, or through action of glutamate dehydrogenase on glutamate.

35 一位新生兒有餵食欠佳、昏睡、呼吸急促症狀，血液檢查發現有輕微的呼吸性鹼中毒（respiratory alkalosis）與高血氨（hyperammonemia）。家族病史分析發現屬於性聯遺傳疾病。下列尿素循環（urea cycle）中何種酵素最可能出現功能異常？

 尿黑酸氧化酶（homogentisate oxygenase）

鳥胺酸轉胺基甲醯基酶（ornithine transcarbamoylase）

胱硫醚合成酶（cystathionine β-synthase）

精胺基琥珀酸酶（argininosuccinase）

ans B OTC

if defective, result in accumulation of urea, then hepatic encephalopathy

The neonatal period

Children with very severe urea cycle disorders typically show symptoms after the first 24 hours of life. The baby may be irritable at first, followed by vomiting and increasing lethargy. Soon after, seizures, hypotonia (poor muscle tone), respiratory distress, and coma may occur. If untreated, the child will die. These symptoms are caused by rising ammonia levels in the blood. Acute neonatal symptoms are most frequently seen in, but not limited to, boys with OTC Deficiency.

The six urea cycle disorders

There are six disorders of the urea cycle. Each is referred to by the initials of the missing enzyme.

Location	Abb.	Enzyme	Disorder	Measurements
Mitochondria	NAGS	N-Acetylglutamate synthetase	N-Acetylglutamate synthase deficiency	+Ammonia
Mitochondria	CPS1	Carbamoyl phosphate synthetase I	Carbamoyl phosphate synthetase I deficiency	+Ammonia
Mitochondria	OTC	Ornithine transcarbamylase	Ornithine transcarbamylase deficiency	+Ornithine, +Uracil, +Orotic acid
Cytosol	ASS	Argininosuccinic acid synthetase	"AS deficiency" or citrullinemia	+Citrulline
Cytosol	ASL	Argininosuccinase acid lyase	"AL deficiency" or argininosuccinic aciduria(ASA)	+Citrulline, +Argininosuccinic acid
Cytosol	ARG	Arginase	"Arginase deficiency" or argininemia	+Arginine

Ornithine transcarbamylase deficiency (OTCD), the most common of the urea cycle disorders, is a rare metabolic disorder, occurring in one out of every 80,000 births. OTCD is agenetic disorder resulting in a mutated and ineffective form of the enzyme ornithine transcarbamylase.