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HMGCS2
3-hydroxy-3-methylglutaryl-CoA synthase 2 (mitochondrial) is an enzyme in humans that is encoded by the ''HMGCS2'' gene. The protein encoded by this gene belongs to the HMG-CoA synthase family. It is a mitochondrial enzyme that catalyzes the second and rate-limiting reaction of ketogenesis, a metabolic pathway that provides lipid-derived energy for various organs during times of carbohydrate deprivation, such as fasting, by addition of a third acetyl group to acetoacetyl-CoA, producing HMG-CoA. Mutations in this gene are associated with HMG-CoA synthase deficiency (also known as HMGCS2D), affecting ketone body synthesis. Alternatively spliced transcript variants encoding different isoforms have been found for this gene. Occurrence HMGCS2 is not found in cetaceans, elephantids, or Old World fruit bats Megabats constitute the family Pteropodidae of the order Chiroptera (bats). They are also called fruit bats, Old World fruit bats, or—especially the genera ''Acer ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Hydroxymethylglutaryl-CoA Synthase
In molecular biology, hydroxymethylglutaryl-CoA synthase or HMG-CoA synthase is an enzyme which catalyzes the reaction in which acetyl-CoA condenses with acetoacetyl-CoA to form 3-hydroxy-3-methylglutaryl-CoA (HMG-CoA). This reaction comprises the second step in the mevalonate-dependent isoprenoid biosynthesis pathway. HMG-CoA is an intermediate in both cholesterol synthesis and ketogenesis. This reaction is overactivated in patients with diabetes mellitus type 1 if left untreated, due to prolonged insulin deficiency and the exhaustion of substrates for gluconeogenesis and the TCA cycle, notably oxaloacetate. This results in shunting of excess acetyl-CoA into the ketone synthesis pathway via HMG-CoA, leading to the development of diabetic ketoacidosis. The 3 substrates of this enzyme are acetyl-CoA, H2O, and acetoacetyl-CoA, whereas its two products are (''S'')-3-hydroxy-3-methylglutaryl-CoA and CoA. In humans, the protein is encoded by the ''HMGCS1'' gene on chromo ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Enzyme
Enzymes () are proteins that act as biological catalysts by accelerating chemical reactions. The molecules upon which enzymes may act are called substrate (chemistry), substrates, and the enzyme converts the substrates into different molecules known as product (chemistry), products. Almost all metabolism, metabolic processes in the cell (biology), cell need enzyme catalysis in order to occur at rates fast enough to sustain life. Metabolic pathways depend upon enzymes to catalyze individual steps. The study of enzymes is called ''enzymology'' and the field of pseudoenzyme, pseudoenzyme analysis recognizes that during evolution, some enzymes have lost the ability to carry out biological catalysis, which is often reflected in their amino acid sequences and unusual 'pseudocatalytic' properties. Enzymes are known to catalyze more than 5,000 biochemical reaction types. Other biocatalysts are Ribozyme, catalytic RNA molecules, called ribozymes. Enzymes' Chemical specificity, specific ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Gene
In biology, the word gene (from , ; "... Wilhelm Johannsen coined the word gene to describe the Mendelian units of heredity..." meaning ''generation'' or ''birth'' or ''gender'') can have several different meanings. The Mendelian gene is a basic unit of heredity and the molecular gene is a sequence of nucleotides in DNA that is transcribed to produce a functional RNA. There are two types of molecular genes: protein-coding genes and noncoding genes. During gene expression, the DNA is first copied into RNA. The RNA can be directly functional or be the intermediate template for a protein that performs a function. The transmission of genes to an organism's offspring is the basis of the inheritance of phenotypic traits. These genes make up different DNA sequences called genotypes. Genotypes along with environmental and developmental factors determine what the phenotypes will be. Most biological traits are under the influence of polygenes (many different genes) as well as g ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Ketogenesis
Ketogenesis is the biochemical process through which organisms produce ketone bodies by breaking down fatty acids and ketogenic amino acids. The process supplies energy to certain organs, particularly the brain, heart and skeletal muscle, under specific scenarios including fasting, caloric restriction, sleep, or others. (In rare metabolic diseases, insufficient gluconeogenesis can cause excessive ketogenesis and hypoglycemia, which may lead to the life-threatening condition known as non-diabetic ketoacidosis.) Ketone bodies are not obligately produced from fatty acids; rather a meaningful amount of them is synthesized only in a situation of carbohydrate and protein insufficiency, where only fatty acids are readily available as fuel for their production. Production Ketone bodies are produced mainly in the mitochondria of liver cells, and synthesis can occur in response to an unavailability of blood glucose, such as during fasting. Other cells, e.g. human astrocytes, are capa ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Transcript Variant
Alternative splicing, or alternative RNA splicing, or differential splicing, is an alternative splicing process during gene expression that allows a single gene to code for multiple proteins. In this process, particular exons of a gene may be included within or excluded from the final, processed messenger RNA (mRNA) produced from that gene. This means the exons are joined in different combinations, leading to different (alternative) mRNA strands. Consequently, the proteins translated from alternatively spliced mRNAs will contain differences in their amino acid sequence and, often, in their biological functions (see Figure). Biologically relevant alternative splicing occurs as a normal phenomenon in eukaryotes, where it increases the number of proteins that can be encoded by the genome. In humans, it is widely believed that ~95% of multi-exonic genes are alternatively spliced to produce functional alternative products from the same gene but many scientists believe that most of ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Cetacea
Cetacea (; , ) is an infraorder of aquatic mammals that includes whales, dolphins, and porpoises. Key characteristics are their fully aquatic lifestyle, streamlined body shape, often large size and exclusively carnivorous diet. They propel themselves through the water with powerful up-and-down movement of their tail which ends in a paddle-like fluke, using their flipper-shaped forelimbs to maneuver. While the majority of cetaceans live in marine environments, a small number exclusively reside in brackish water or fresh water. Having a cosmopolitan distribution, they can be found in some rivers and all of Earth's oceans, and many species inhabit vast ranges where they migrate with the changing of the seasons. Cetaceans are famous for their high intelligence and complex social behaviour as well as for the enormous size of some of the group's members, such as the blue whale which reaches a maximum confirmed length of 29.9 meters (98 feet) and a weight of 173 tonnes (190 short ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Elephantidae
Elephantidae is a family of large, herbivorous proboscidean mammals collectively called elephants and mammoths. These are terrestrial large mammals with a snout modified into a trunk and teeth modified into tusks. Most genera and species in the family are extinct. Only two genera, '' Loxodonta'' (African elephants) and ''Elephas'' (Asiatic elephants), are living. The family was first described by John Edward Gray in 1821, and later assigned to taxonomic ranks within the order Proboscidea. Elephantidae has been revised by various authors to include or exclude other extinct proboscidean genera. Classification Scientific classification of Elephantidae taxa embraces an extensive record of fossil specimens, over millions of years, some of which existed until the end of the last ice age. Some species were extirpated more recently. The discovery of new specimens and proposed cladistics have resulted in systematic revisions of the family and related proboscideans. Elephantids are ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Megabat
Megabats constitute the family Pteropodidae of the order Chiroptera ( bats). They are also called fruit bats, Old World fruit bats, or—especially the genera '' Acerodon'' and '' Pteropus''— flying foxes. They are the only member of the superfamily Pteropodoidea, which is one of two superfamilies in the suborder Yinpterochiroptera. Internal divisions of Pteropodidae have varied since subfamilies were first proposed in 1917. From three subfamilies in the 1917 classification, six are now recognized, along with various tribes. As of 2018, 197 species of megabat had been described. The leading theory of the evolution of megabats has been determined primarily by genetic data, as the fossil record for this family is the most fragmented of all bats. They likely evolved in Australasia, with the common ancestor of all living pteropodids existing approximately 31 million years ago. Many of their lineages probably originated in Melanesia, then dispersed over time to mainland Asi ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
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