hawkinsin: structure [Medical Subject Headings (MeSH), National Library of Medicine, extracted Dec-2023]
hawkinsin : A cysteine derivative that is cysteine in which the thiol group is substituted by a [2-(carboxymethyl)-2,5-dihydroxycyclohex-3-en-1-yl]sulfanediyl group. Hawkinsinuria is an inherited disorder which is characterized by the inability to break down the amino acid tyrosine. This results in the finding of certain metabolites in the urine, such as hawkinsin. [Chemical Entities of Biological Interest (ChEBI), Hastings J, Owen G, Dekker A, Ennis M, Kale N, Muthukrishnan V, Turner S, Swainston N, Mendes P, Steinbeck C. (2016). ChEBI in 2016: Improved services and an expanding collection of metabolites. Nucleic Acids Res]
| ID Source | ID |
|---|---|
| PubMed CID | 173909 |
| CHEBI ID | 149584 |
| SCHEMBL ID | 1660794 |
| MeSH ID | M0063197 |
| Synonym |
|---|
| hawkinsin |
| s-[2-(carboxymethyl)-2,5-dihydroxycyclohex-3-en-1-yl]cysteine |
| 63224-90-8 |
| CHEBI:149584 |
| 2-cyclohexene-1-acetic acid, 6-((2-amino-2-carboxyethyl)thio)-1,4-dihydroxy- |
| (2-l-cystein-s-yl-1,4-dihydroxycyclohex-5-en-1-yl)acetic acid |
| SCHEMBL1660794 |
| 2-amino-3-{[2-(carboxymethyl)-2,5-dihydroxycyclohex-3-en-1-yl]sulfanyl}propanoic acid |
| Q5685191 |
| DTXSID10866992 |
| 2-amino-3-[[2-(carboxymethyl)-2,5-dihydroxy-1-cyclohex-3 enyl]sulfanyl]propanoic acid |
Hawkinsinuria is a rarely diagnosed autosomal dominantly transmitted inborn error of tyrosine metabolism. Hawkinsin is a sulfur amino acid identified as (2-l-cystein-S-yl, 4-dihydroxycyclohex-5-en-1-yl)acetic acid.
| Excerpt | Reference | Relevance |
|---|---|---|
| "Hawkinsinuria is a rarely diagnosed autosomal dominantly transmitted inborn error of tyrosine metabolism with impaired conversion of 4-hydroxyphenylpyruvate to homogentisate. " | ( Long-term follow up of a new case of hawkinsinuria. Engelke, U; Lehnert, W; Stögmann, W; van den Berg, GB; Wevers, RA, 1999) | 2.02 |
| "Hawkinsin is a sulfur amino acid identified as (2-l-cystein-S-yl, 4-dihydroxycyclohex-5-en-1-yl)acetic acid." | ( Mutations in the 4-hydroxyphenylpyruvic acid dioxygenase gene are responsible for tyrosinemia type III and hawkinsinuria. Awata, H; Boneh, A; Danks, DM; Endo, F; Matsuda, I; Matsuura, T; Milovac, T; Ploechl, E; Scott, CR; Tomoeda, K, 2000) | 1.24 |
| Role | Description |
|---|---|
| biomarker | A substance used as an indicator of a biological state. |
| human urinary metabolite | Any metabolite (endogenous or exogenous) found in human urine samples. |
| [role information is derived from Chemical Entities of Biological Interest (ChEBI), Hastings J, Owen G, Dekker A, Ennis M, Kale N, Muthukrishnan V, Turner S, Swainston N, Mendes P, Steinbeck C. (2016). ChEBI in 2016: Improved services and an expanding collection of metabolites. Nucleic Acids Res] | |
| Class | Description |
|---|---|
| dicarboxylic acid | Any carboxylic acid containing two carboxy groups. |
| cysteine derivative | An amino acid derivative resulting from reaction of cysteine at the amino group, carboxy group, or thiol group, or from the replacement of any hydrogen of cysteine by a heteroatom. The definition normally excludes peptides containing cysteine residues. |
| cycloalkene | An unsaturated monocyclic hydrocarbon having at least one endocyclic double bond. |
| diol | A compound that contains two hydroxy groups, generally assumed to be, but not necessarily, alcoholic. Aliphatic diols are also called glycols. |
| tertiary allylic alcohol | An allylic alcohol in which the carbon atom that links the double bond to the hydroxy group is also attached to two other carbons (R4,R5 =/= H). |
| secondary allylic alcohol | An allylic alcohol in which the carbon atom that links the double bond to the hydroxy group is also attached to one other carbon and one hydrogen. |
| [compound class information is derived from Chemical Entities of Biological Interest (ChEBI), Hastings J, Owen G, Dekker A, Ennis M, Kale N, Muthukrishnan V, Turner S, Swainston N, Mendes P, Steinbeck C. (2016). ChEBI in 2016: Improved services and an expanding collection of metabolites. Nucleic Acids Res] | |
| Pathway | Proteins | Compounds |
|---|---|---|
| Tyrosine metabolism and related disorders | 7 | 24 |
| Timeframe | Studies, This Drug (%) | All Drugs % |
|---|---|---|
| pre-1990 | 5 (50.00) | 18.7374 |
| 1990's | 3 (30.00) | 18.2507 |
| 2000's | 2 (20.00) | 29.6817 |
| 2010's | 0 (0.00) | 24.3611 |
| 2020's | 0 (0.00) | 2.80 |
| [information is prepared from research data collected from National Library of Medicine (NLM), extracted Dec-2023] | ||
According to the monthly volume, diversity, and competition of internet searches for this compound, as well the volume and growth of publications, there is estimated to be moderate demand-to-supply ratio for research on this compound.
| This Compound (22.33) All Compounds (24.57) |
| Publication Type | This drug (%) | All Drugs (%) |
|---|---|---|
| Trials | 0 (0.00%) | 5.53% |
| Reviews | 1 (10.00%) | 6.00% |
| Case Studies | 5 (50.00%) | 4.05% |
| Observational | 0 (0.00%) | 0.25% |
| Other | 4 (40.00%) | 84.16% |
| [information is prepared from research data collected from National Library of Medicine (NLM), extracted Dec-2023] | ||