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Matrix Gla Protein
Matrix Gla protein (MGP) is member of a family of vitamin K2 dependent, Gla-containing proteins. MGP has a high affinity binding to calcium ions, similar to other Gla-containing proteins. The protein acts as an inhibitor of vascular mineralization and plays a role in bone organization. MGP is found in a number of body tissues in mammals, birds, and fish. Its mRNA is present in bone, cartilage, heart, and kidney. It is present in bone together with the related vitamin K2-dependent protein osteocalcin. In bone, its production is increased by vitamin D. Genetics The ''MGP'' was linked to the short arm of chromosome 12 in 1990. Its mRNA sequence length is 585 bases long in humans. Physiology MGP and osteocalcin are both calcium-binding proteins that may participate in the organisation of bone tissue. Both have glutamate residues that are post-translationally carboxylated by the enzyme gamma-glutamyl carboxylase in a reaction that requires Vitamin K hydroquinone. Role in ...
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Vitamin K2
Vitamin K2 or menaquinone (MK) () is one of three types of vitamin K, the other two being vitamin K1 ( phylloquinone) and K3 (menadione). K2 is both a tissue and bacterial product (derived from vitamin K1 in both cases) and is usually found in animal products or fermented foods. The number ''n'' of isoprenyl units in their side chain differs and ranges from 4 to 13, hence vitamin K2 consists of various forms. It is indicated as a suffix (-n), e. g. MK-7 or MK-9. * The most common in the human diet is the short-chain, water-soluble menatetrenone (MK-4), which is commonly found in animal products. However, at least one published study concluded that "MK-4 present in food does not contribute to the vitamin K status as measured by serum vitamin K levels." The MK-4 in animal (including human) tissue is made from dietary plant vitamin K1. This process can be accomplished by animal tissues alone, as it proceeds in germ-free rodents. * Long-chain menaquinones (longer than MK-4) include ...
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Post-translational Modification
In molecular biology, post-translational modification (PTM) is the covalent process of changing proteins following protein biosynthesis. PTMs may involve enzymes or occur spontaneously. Proteins are created by ribosomes, which translation (biology), translate mRNA into polypeptide chains, which may then change to form the mature protein product. PTMs are important components in cell signal transduction, signalling, as for example when prohormones are converted to hormones. Post-translational modifications can occur on the amino acid side chains or at the protein's C-terminus, C- or N-terminus, N- termini. They can expand the chemical set of the 22 proteinogenic amino acid, amino acids by changing an existing functional group or adding a new one such as phosphate. Phosphorylation is highly effective for controlling the enzyme activity and is the most common change after translation. Many eukaryotic and prokaryotic proteins also have carbohydrate molecules attached to them in a pro ...
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Extracellular Matrix Proteins
In biology, the extracellular matrix (ECM), also called intercellular matrix (ICM), is a network consisting of extracellular macromolecules and minerals, such as collagen, enzymes, glycoproteins and hydroxyapatite that provide structural and biochemical support to surrounding cells. Because multicellularity evolved independently in different multicellular lineages, the composition of ECM varies between multicellular structures; however, cell adhesion, cell-to-cell communication and differentiation are common functions of the ECM. The animal extracellular matrix includes the interstitial matrix and the basement membrane. Interstitial matrix is present between various animal cells (i.e., in the intercellular spaces). Gels of polysaccharides and fibrous proteins fill the interstitial space and act as a compression buffer against the stress placed on the ECM. Basement membranes are sheet-like depositions of ECM on which various epithelial cells rest. Each type of connective tissu ...
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Arterial
An artery () is a blood vessel in humans and most other animals that takes oxygenated blood away from the heart in the systemic circulation to one or more parts of the body. Exceptions that carry deoxygenated blood are the pulmonary arteries in the pulmonary circulation that carry blood to the lungs for oxygenation, and the umbilical arteries in the fetal circulation that carry deoxygenated blood to the placenta. It consists of a multi-layered artery wall wrapped into a tube-shaped channel. Arteries contrast with veins, which carry deoxygenated blood back towards the heart; or in the pulmonary and fetal circulations carry oxygenated blood to the lungs and fetus respectively. Structure The anatomy of arteries can be separated into gross anatomy, at the macroscopic level, and microanatomy, which must be studied with a microscope. The arterial system of the human body is divided into systemic arteries, carrying blood from the heart to the whole body, and pulmonary arteries, ...
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Hypoplasia
Hypoplasia (; adjective form ''hypoplastic'') is underdevelopment or incomplete development of a tissue or organ.Definition: hypoplasia
Dictionary of Cell and Molecular Biology (11 March 2008) Although the term is not always used precisely, it properly refers to an inadequate or below-normal number of cells.Hypoplasia
Stedman's Medical Dictionary. lww.com
Hypoplasia is similar to aplasia, but less severe. It is technically ''not'' the opposite of
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Pulmonary Artery
A pulmonary artery is an artery in the pulmonary circulation that carries deoxygenated blood from the right side of the heart to the lungs. The largest pulmonary artery is the ''main pulmonary artery'' or ''pulmonary trunk'' from the heart, and the smallest ones are the arterioles, which lead to the capillaries that surround the pulmonary alveoli. Structure The pulmonary arteries are blood vessels that carry systemic venous blood from the right ventricle of the heart to the microcirculation of the lungs. Unlike in other organs where arteries supply oxygenated blood, the blood carried by the pulmonary arteries is deoxygenated, as it is venous blood returning to the heart. The main pulmonary arteries emerge from the right side of the heart and then split into smaller arteries that progressively divide and become arterioles, eventually narrowing into the capillary microcirculation of the lungs where gas exchange occurs. Pulmonary trunk In order of blood flow, the pulmonary ...
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Stenosis
Stenosis () is the abnormal narrowing of a blood vessel or other tubular organ or structure such as foramina and canals. It is also sometimes called a stricture (as in urethral stricture). ''Stricture'' as a term is usually used when narrowing is caused by contraction of smooth muscle (e.g. achalasia, prinzmetal angina); ''stenosis'' is usually used when narrowing is caused by lesion that reduces the space of lumen (e.g. atherosclerosis). The term coarctation is another synonym, but is commonly used only in the context of aortic coarctation. Restenosis is the recurrence of stenosis after a procedure. Examples Examples of vascular stenotic lesions include: * Intermittent claudication (peripheral artery stenosis) * Angina ( coronary artery stenosis) * Carotid artery stenosis which predispose to (strokes and transient ischaemic episodes) * Renal artery stenosis Types In heart valves The types of stenoses in heart valves are: * Pulmonary valve stenosis, which is th ...
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Peripheral
A peripheral device, or simply peripheral, is an auxiliary hardware device that a computer uses to transfer information externally. A peripheral is a hardware component that is accessible to and controlled by a computer but is not a core component of the computer. A peripheral can be categorized based on the direction in which information flows relative to the computer: * The computer receives data from an '' input device''; examples: mouse, keyboard, scanner, game controller, microphone and webcam * The computer sends data to an '' output device''; examples: monitor, printer, headphones, and speakers * The computer sends and receives data via an ''input/output device''; examples: storage device (such as disk drive, solid-state drive, USB flash drive, memory card and tape drive), modem, router, gateway and network adapter Many modern electronic devices, such as Internet-enabled digital watches, video game consoles, smartphones, and tablet computers, have in ...
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Cartilage
Cartilage is a resilient and smooth type of connective tissue. Semi-transparent and non-porous, it is usually covered by a tough and fibrous membrane called perichondrium. In tetrapods, it covers and protects the ends of long bones at the joints as articular cartilage, and is a structural component of many body parts including the rib cage, the neck and the bronchial tubes, and the intervertebral discs. In other taxa, such as chondrichthyans and cyclostomes, it constitutes a much greater proportion of the skeleton. It is not as hard and rigid as bone, but it is much stiffer and much less flexible than muscle. The matrix of cartilage is made up of glycosaminoglycans, proteoglycans, collagen fibers and, sometimes, elastin. It usually grows quicker than bone. Because of its rigidity, cartilage often serves the purpose of holding tubes open in the body. Examples include the rings of the trachea, such as the cricoid cartilage and carina. Cartilage is composed of specialized c ...
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Keutel Syndrome
Keutel syndrome (KS) is a rare autosomal recessive genetic disorder characterized by abnormal diffuse cartilage calcification, hypoplasia of the mid-face, peripheral pulmonary stenosis, hearing loss, short distal phalanges (tips) of the fingers and mild mental retardation. Individuals with KS often present with peripheral pulmonary stenosis, brachytelephalangism, sloping forehead, midface hypoplasia, and receding chin. It is associated with abnormalities in the gene coding for matrix gla protein, ''MGP''. Being an autosomal recessive disorder, it may be inherited from two unaffected, abnormal MGP-carrying parents. Thus, people who inherit two affected ''MGP'' alleles will likely inherit KS. It was first identified in 1972 as a novel rare genetic disorder sharing similar symptoms with chondrodysplasia punctata. Multiple forms of chondrodysplasia punctata share symptoms consistent with KS including abnormal cartilage calcification, forceful respiration, brachytelephalangism, hypoto ...
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Vitamin K
Vitamin K is a family of structurally similar, fat-soluble vitamers found in foods and marketed as dietary supplements. The human body requires vitamin K for post-translational modification, post-synthesis modification of certain proteins that are required for blood coagulation ("K" from Danish ''koagulation'', for "coagulation") and for controlling molecular binding, binding of calcium in bones and other tissue (biology), tissues. The complete synthesis involves final modification of these "Gla proteins" by the enzyme gamma-glutamyl carboxylase that uses vitamin K as a cofactor (biochemistry), cofactor. Vitamin K is used in the liver as the intermediate VKH2 to deprotonate a glutamate residue and then is reprocessed into vitamin K through a vitamin K oxide intermediate. The presence of uncarboxylated proteins indicates a vitamin K deficiency. Carboxylation allows them to bind (chelate) calcium ions, which they cannot do otherwise. Without vitamin  ...
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Gamma-glutamyl Carboxylase
Gamma-glutamyl carboxylase is an enzyme that in humans is encoded by the ''GGCX'' gene, located on chromosome 2 at 2p12. Function Gamma-glutamyl carboxylase is an enzyme that catalyzes the posttranslational modification of vitamin K-dependent proteins. Many of these vitamin K-dependent proteins are involved in coagulation so the function of the encoded enzyme is essential for hemostasis. Most gla domain-containing proteins depend on this carboxylation reaction for posttranslational modification. In humans, the gamma-glutamyl carboxylase enzyme is most highly expressed in the liver. Catalytic reaction Gamma-glutamyl carboxylase oxidizes vitamin K hydroquinone to Vitamin K-2,3-epoxide, while simultaneously adding CO2 to protein-bound glutamic acid (abbreviation = Glu) to form gamma-carboxyglutamic acid (also called gamma-carboxyglutamate, abbreviation = Gla). Presence of two carboxylate groups causes chelation of Ca2+, resulting in change in tertiary structure of protein and its ...
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