Osteolathyrism is a collagen

Collagen

Collagen is a group of naturally occurring proteins found in animals, especially in the flesh and connective tissues of mammals. It is the main component of connective tissue, and is the most abundant protein in mammals, making up about 25% to 35% of the whole-body protein content...

 cross-linking deficiency

Deficiency

A deficiency is generally a lack of something. It may also refer to:*A deficient number, in mathematics, a number n for which σ A deficiency is generally a lack of something. It may also refer to:...

 brought on by dietary over-reliance on the seeds of Lathyrus sativus

Lathyrus sativus

Lathyrus sativus, is a legume commonly grown for human consumption and livestock feed in Asia and East Africa. It is a particularly important crop in areas that are prone to drought and famine, and is thought of as an 'insurance crop' as it produces reliable yields when all other crops...

. It is commonly seen in combination with neurolathyrism and angiolathyrism

Angiolathyrism

Angio = Blood vessel, Lathyrism = disease due to Lathyrus Sativus. It is commonly associated with Osteolathyrism and Neurolathyrism and is caused by over consumption of Lathyrus sativus, also known as sweet pea. Main chemical responsible is β-Aminopropionitrile which prevents collagen cross-linking...

 in impoverished areas where famine

Famine

A famine is a widespread scarcity of food, caused by several factors including crop failure, overpopulation, or government policies. This phenomenon is usually accompanied or followed by regional malnutrition, starvation, epidemic, and increased mortality. Every continent in the world has...

 demands reliance on a crop

Crop

Crop may refer to:* Crop, a plant grown and harvested for agricultural use* Crop , part of the alimentary tract of some animals* Crop , a modified whip used in horseback riding or disciplining humans...

 with known detrimental effects. Osteolathyrism is caused by a variety of osteolathyrogenic compounds, specifically excitatory amino-compounds. The most widely-studied of these compounds is beta-aminopropionitrile (BAPN), which exerts its deleterious effect by an unknown yet potently irreversible mechanism. Other instigators are ureides, semicarbazide

Semicarbazide

In organic chemistry, semicarbazide is a derivative of urea, where NH2 on one side has been replaced with H2NNH2 hydrazine, yielding H2NNHCNH2.Semicarbazide is used as a detection reagent on thin layer chromatography...

s and thiosemicarbazides, which are believed to chelate the prosthetic Cu(II)

Copper

Copper is a chemical element with the symbol Cu and atomic number 29. It is a ductile metal with very high thermal and electrical conductivity. Pure copper is soft and malleable; an exposed surface has a reddish-orange tarnish...

-bipyridine

Bipyridine

Bipyridines are a family of chemical compounds with the formula 2, which are formed by the coupling of two pyridine rings. Six isomers of bipyridine exist, but two isomers are prominent: 2,2'-bipyridine is a popular ligand in coordination chemistry and 4,4'-bipyridine is a precursor to the...

 cofactor

Cofactor (biochemistry)

A cofactor is a non-protein chemical compound that is bound to a protein and is required for the protein's biological activity. These proteins are commonly enzymes, and cofactors can be considered "helper molecules" that assist in biochemical transformations....

 complex in the enzyme

Enzyme

Enzymes are proteins that catalyze chemical reactions. In enzymatic reactions, the molecules at the beginning of the process, called substrates, are converted into different molecules, called products. Almost all chemical reactions in a biological cell need enzymes in order to occur at rates...

 lysyl oxidase

Lysyl oxidase

Lysyl oxidase also known as protein-lysine 6-oxidase is a protein that, in humans, is encoded by the LOX gene. Its inhibition can cause lathyrism, but, at the same time, its upregulation by tumor cells may promote metastasis of the existing tumor, causing it to become malignant and cancerous.-...

.

Lysyl oxidase is an important enzyme for the creation of crosslinks between collagen

Collagen

Collagen is a group of naturally occurring proteins found in animals, especially in the flesh and connective tissues of mammals. It is the main component of connective tissue, and is the most abundant protein in mammals, making up about 25% to 35% of the whole-body protein content...

 triple-helices in connective tissue

Connective tissue

"Connective tissue" is a fibrous tissue. It is one of the four traditional classes of tissues . Connective Tissue is found throughout the body.In fact the whole framework of the skeleton and the different specialized connective tissues from the crown of the head to the toes determine the form of...

. By oxidizing the terminal amino group of lysine

Lysine

Lysine is an α-amino acid with the chemical formula HO2CCH4NH2. It is an essential amino acid, which means that the human body cannot synthesize it. Its codons are AAA and AAG....

, an aldehyde

Aldehyde

An aldehyde is an organic compound containing a formyl group. This functional group, with the structure R-CHO, consists of a carbonyl center bonded to hydrogen and an R group....

 is created. This aldehyde can undergo several reactions with neighboring aldehydes or amine

Amine

Amines are organic compounds and functional groups that contain a basic nitrogen atom with a lone pair. Amines are derivatives of ammonia, wherein one or more hydrogen atoms have been replaced by a substituent such as an alkyl or aryl group. Important amines include amino acids, biogenic amines,...

s to create strong covalent cross-links between collagen tertiary structure

Tertiary structure

In biochemistry and molecular biology, the tertiary structure of a protein or any other macromolecule is its three-dimensional structure, as defined by the atomic coordinates.-Relationship to primary structure:...

s in bone

Bone

Bones are rigid organs that constitute part of the endoskeleton of vertebrates. They support, and protect the various organs of the body, produce red and white blood cells and store minerals. Bone tissue is a type of dense connective tissue...

 and cartilage

Cartilage

Cartilage is a flexible connective tissue found in many areas in the bodies of humans and other animals, including the joints between bones, the rib cage, the ear, the nose, the elbow, the knee, the ankle, the bronchial tubes and the intervertebral discs...

. The main product of these reactions is the aldimine

Aldimine

In organic chemistry, an aldimine is an imine that is an analog of an aldehyde.As such, aldimines have the general formula R–CH=N–R. Aldimines are similar to ketimines, which are analogs of ketones.An important subset of aldimines are the Schiff bases,...

 compound dehydrohyroxylysinonorleucine.

This unique crosslink can be formed by the Schiff base

Schiff base

A Schiff base, named after Hugo Schiff, is a compound with a functional group that contains a carbon-nitrogen double bond with the nitrogen atom connected to an aryl or alkyl group, not hydrogen....

 mechanism in which the lone pair of electron

Electron

The electron is a subatomic particle with a negative elementary electric charge. It has no known components or substructure; in other words, it is generally thought to be an elementary particle. An electron has a mass that is approximately 1/1836 that of the proton...

s on a primary amine react with the carbonyl

Carbonyl

In organic chemistry, a carbonyl group is a functional group composed of a carbon atom double-bonded to an oxygen atom: C=O. It is common to several classes of organic compounds, as part of many larger functional groups....

 carbon of an aldehyde. Other crosslinks include the formation of an α,β-unsaturated

Saturation (chemistry)

In chemistry, saturation has six different meanings, all based on reaching a maximum capacity...

 ketone

Ketone

In organic chemistry, a ketone is an organic compound with the structure RCR', where R and R' can be a variety of atoms and groups of atoms. It features a carbonyl group bonded to two other carbon atoms. Many ketones are known and many are of great importance in industry and in biology...

 via aldol condensation

Aldol condensation

An aldol condensation is an organic reaction in which an enol or an enolate ion reacts with a carbonyl compound to form a β-hydroxyaldehyde or β-hydroxyketone, followed by a dehydration to give a conjugated enone....

 and hydroxylysinonorleucine.

If these crosslinks are not formed, as in the case of osteolathyrism, the synthesis of strong mesenchymal and mesodermal tissue is inhibited. Symptoms of osteolathyrism include weakness and fragility of connective tissue (i.e., skin, bones, and blood vessels (angiolathyrism

Angiolathyrism

Angio = Blood vessel, Lathyrism = disease due to Lathyrus Sativus. It is commonly associated with Osteolathyrism and Neurolathyrism and is caused by over consumption of Lathyrus sativus, also known as sweet pea. Main chemical responsible is β-Aminopropionitrile which prevents collagen cross-linking...

) and the paralysis

Paralysis

Paralysis is loss of muscle function for one or more muscles. Paralysis can be accompanied by a loss of feeling in the affected area if there is sensory damage as well as motor. A study conducted by the Christopher & Dana Reeve Foundation, suggests that about 1 in 50 people have been diagnosed...

 of the lower extremities associated with neurolathyrism. For these reasons, compounds containing lathyrogens should be avoided during pregnancy

Pregnancy

Pregnancy refers to the fertilization and development of one or more offspring, known as a fetus or embryo, in a woman's uterus. In a pregnancy, there can be multiple gestations, as in the case of twins or triplets...

 and growth of a child

Child

Biologically, a child is generally a human between the stages of birth and puberty. Some vernacular definitions of a child include the fetus, as being an unborn child. The legal definition of "child" generally refers to a minor, otherwise known as a person younger than the age of majority...

.