Palmitoylcarnitine

Palmitoylcarnitine
Names
	IUPAC name 3-(palmitoyloxy)-4-(trimethylammonio)butanoate
Identifiers
CAS Number	1935-18-8 ;
3D model (JSmol)	Interactive image;
ChEMBL	ChEMBL301722 ;
ChemSpider	448 ;
MeSH	Palmitoylcarnitine
PubChem CID	461;
UNII	U8K6DKA8V2 ;
CompTox Dashboard (EPA)	DTXSID90895028 ;
	InChI InChI=1S/C23H45NO4/c1-5-6-7-8-9-10-11-12-13-14-15-16-17-18-23(27)28-21(19-22(25)26)20-24(2,3)4/h21H,5-20H2,1-4H3 Key: XOMRRQXKHMYMOC-UHFFFAOYSA-N ; InChI=1/C23H45NO4/c1-5-6-7-8-9-10-11-12-13-14-15-16-17-18-23(27)28-21(19-22(25)26)20-24(2,3)4/h21H,5-20H2,1-4H3 Key: XOMRRQXKHMYMOC-UHFFFAOYAU;
	SMILES [O-]C(=O)CC(OC(=O)CCCCCCCCCCCCCCC)C[N+](C)(C)C;
Properties
Chemical formula	C23H45NO4
Molar mass	399.608 g/mol
	Except where otherwise noted, data are given for materials in their standard state (at 25 °C [77 °F], 100 kPa). verify (what is ?) Infobox references

Palmitoylcarnitine is an ester derivative of carnitine involved in the metabolism of fatty acids. During the tricarboxylic acid cycle (TCA), fatty acids undergo a process known as β-oxidation to produce energy in the form of ATP. β-oxidation occurs primarily within mitochondria, however the mitochondrial membrane prevents the entry of long chain fatty acids (>C10), so the conversion of fatty acids such as palmitic acid is key.^[1] Palmitic acid is brought to the cell and once inside the cytoplasm is first converted to Palmitoyl-CoA. Palmitoyl-CoA has the ability to freely pass the outer mitochondrial membrane, but the inner membrane is impermeable to the Acyl-CoA and thioester forms of various long-chain fatty acids such as palmitic acid. The palmitoyl-CoA is then enzymatically transformed into palmitoylcarnitine via the Carnitine O-palmitoyltransferase family. The palmitoylcarnitine is then actively transferred into the inner membrane of the mitochondria via the carnitine-acylcarnitine translocase.^[2] Once inside the inner mitochondrial membrane, the same Carnitine O-palmitoyltransferase family is then responsible for transforming the palmitoylcarnitine back to the palmitoyl-CoA form.

Structure

Chemical Structure of Palmitic Acid

Palmitoylcarnitine contains the saturated fatty acid known as palmitic acid (C16:0) which is bound to the β-hydroxy group of the carnitine. The core carnitine structure, consisting of butanoate with a quaternary ammonium attached to C4 and hydroxy group at C3, is a common molecular backbone for the transfer of multiple long chain fatty acids in the TCA cycle.

Function

Energy Generation

Palmitoylcarnitine is one molecule in a family of ester derivatives of carnitine that are utilized in the TCA cycle to generate energy. The beta oxidation yields 7 NADH, 7 FADH2, and 8 Acetyl-CoA chains. This Acetyl-CoA generates 3 NADH, 1 FADH2, and 1 GTP for every molecule in the TCA cycle. Each NADH generates 2.5 ATP in the ETC and FADH2 generates 1.5 ATP. This totals to 108 ATP, but 2 ATP are consumed to generate the initial Palmitoyl-CoA, leaving a net gain of 106 ATP.^{[citation needed]}

Clinical Significance

Palmitoylcarnitine has demonstrated potential as a diagnostic marker in newborns for the medical condition of primary carnitine deficiency.^[3]

Levels of palmitoylcarnitine (palcar) demonstrated significant correlation with dihydrotestosterone (DHT) and its effects in prostate cancer models, suggesting a similar role between the two molecules.^[4]

References

^ Tein, Ingrid (2015-01-01), Darras, Basil T.; Jones, H. Royden; Ryan, Monique M.; De Vivo, Darryl C. (eds.), "Chapter 40 - Lipid Storage Myopathies Due to Fatty Acid Oxidation Defects", Neuromuscular Disorders of Infancy, Childhood, and Adolescence (Second Edition), San Diego: Academic Press, pp. 761–795, doi:10.1016/b978-0-12-417044-5.00040-8, ISBN 978-0-12-417044-5
^ Pande, S. V. (1975-03-01). "A mitochondrial carnitine acylcarnitine translocase system". Proceedings of the National Academy of Sciences. 72 (3): 883–887. Bibcode:1975PNAS...72..883P. doi:10.1073/pnas.72.3.883. ISSN 0027-8424. PMC 432425. PMID 1055387.
^ Huang, Y. L.; Tang, C. F.; Liu, S. C.; Sheng, H. Y.; Tang, F.; Jiang, X.; Zheng, R. D.; Mei, H. F.; Liu, L. (2020-06-02). "[Newborn screening for primary carnitine deficiency and variant spectrum of SLC22A5 gene in Guangzhou]". Zhonghua Er Ke Za Zhi = Chinese Journal of Pediatrics. 58 (6): 476–481. doi:10.3760/cma.j.cn112140-20200323-00292. ISSN 0578-1310. PMID 32521959.
^ Al‐Bakheit, Ala'a; Traka, Maria; Saha, Shikha; Mithen, Richard; Melchini, Antonietta (2016-10-01). "Accumulation of Palmitoylcarnitine and Its Effect on Pro‐Inflammatory Pathways and Calcium Influx in Prostate Cancer". The Prostate. 76 (14): 1326–1337. doi:10.1002/pros.23222. ISSN 0270-4137. PMC 4996340. PMID 27403764.

This biochemistry article is a stub. You can help Wikipedia by expanding it.

[1] Tein, Ingrid (2015-01-01), Darras, Basil T.; Jones, H. Royden; Ryan, Monique M.; De Vivo, Darryl C. (eds.), "Chapter 40 - Lipid Storage Myopathies Due to Fatty Acid Oxidation Defects", Neuromuscular Disorders of Infancy, Childhood, and Adolescence (Second Edition), San Diego: Academic Press, pp. 761–795, doi:10.1016/b978-0-12-417044-5.00040-8, ISBN 978-0-12-417044-5

[2] Pande, S. V. (1975-03-01). "A mitochondrial carnitine acylcarnitine translocase system". Proceedings of the National Academy of Sciences. 72 (3): 883–887. Bibcode:1975PNAS...72..883P. doi:10.1073/pnas.72.3.883. ISSN 0027-8424. PMC 432425. PMID 1055387.

[3] Huang, Y. L.; Tang, C. F.; Liu, S. C.; Sheng, H. Y.; Tang, F.; Jiang, X.; Zheng, R. D.; Mei, H. F.; Liu, L. (2020-06-02). "[Newborn screening for primary carnitine deficiency and variant spectrum of SLC22A5 gene in Guangzhou]". Zhonghua Er Ke Za Zhi = Chinese Journal of Pediatrics. 58 (6): 476–481. doi:10.3760/cma.j.cn112140-20200323-00292. ISSN 0578-1310. PMID 32521959.

[4] Al‐Bakheit, Ala'a; Traka, Maria; Saha, Shikha; Mithen, Richard; Melchini, Antonietta (2016-10-01). "Accumulation of Palmitoylcarnitine and Its Effect on Pro‐Inflammatory Pathways and Calcium Influx in Prostate Cancer". The Prostate. 76 (14): 1326–1337. doi:10.1002/pros.23222. ISSN 0270-4137. PMC 4996340. PMID 27403764.

[1]

[2]

[3]

[4]