Isovalerylcarnitine

3-Methylbutyrylcarnitine, (+-)-isomer

Formula: C12H23NO4 (245.1627)
Chinese Name: 3-甲基丁酰卡尼汀, 异戊酰基肉碱
BioDeep ID: BioDeep_00000013676 ( View LC/MS Profile)
SMILES: CC(C)CC(=O)OC(CC(=O)[O-])C[N+](C)(C)C



Found 11 Sample Hits

m/z Adducts Species Organ Scanning Sample
246.1702 [M+H]+
PPM:0.9
Marker Pen NA DESI (None)
3ul_0.8Mpa_RAW_20241016-PAPER PNMK - MEMI_test
Resolution: 30μm, 315x42

Description

By writing the four English letters “PNMK” on white paper with a marker pen, and then scanning with a DESI ion source to obtain the scanning result. The signal of the chemical substances on the marker pen used appears on the channel with an m/z value of 322.1918, 323.1953, 546.4010, and etc, from the single cell deconvolution sampling layer class_4. This test data was tested by chuxiaoping from PANOMIX’s R&D laboratory.

284.2584 [M+K]+
PPM:7.2
Homo sapiens esophagus DESI ()
LNTO22_1_3 - MTBLS385
Resolution: 75μm, 121x68

Description

210.1496 [M+H-2H2O]+
PPM:3.6
Homo sapiens esophagus DESI ()
LNTO29_16_2 - MTBLS385
Resolution: 17μm, 95x101

Description

210.1493 [M+H-2H2O]+
PPM:2.2
Mus musculus Liver MALDI (CHCA)
Salmonella_final_pos_recal - MTBLS2671
Resolution: 17μm, 691x430

Description

A more complete and holistic view on host–microbe interactions is needed to understand the physiological and cellular barriers that affect the efficacy of drug treatments and allow the discovery and development of new therapeutics. Here, we developed a multimodal imaging approach combining histopathology with mass spectrometry imaging (MSI) and same section imaging mass cytometry (IMC) to study the effects of Salmonella Typhimurium infection in the liver of a mouse model using the S. Typhimurium strains SL3261 and SL1344. This approach enables correlation of tissue morphology and specific cell phenotypes with molecular images of tissue metabolism. IMC revealed a marked increase in immune cell markers and localization in immune aggregates in infected tissues. A correlative computational method (network analysis) was deployed to find metabolic features associated with infection and revealed metabolic clusters of acetyl carnitines, as well as phosphatidylcholine and phosphatidylethanolamine plasmalogen species, which could be associated with pro-inflammatory immune cell types. By developing an IMC marker for the detection of Salmonella LPS, we were further able to identify and characterize those cell types which contained S. Typhimurium. [dataset] Nicole Strittmatter. Holistic Characterization of a Salmonella Typhimurium Infection Model Using Integrated Molecular Imaging, metabolights_dataset, V1; 2022. https://www.ebi.ac.uk/metabolights/MTBLS2671.

246.1702 [M+H]+
PPM:0.9
Mus musculus Liver MALDI (CHCA)
Salmonella_final_pos_recal - MTBLS2671
Resolution: 17μm, 691x430

Description

A more complete and holistic view on host–microbe interactions is needed to understand the physiological and cellular barriers that affect the efficacy of drug treatments and allow the discovery and development of new therapeutics. Here, we developed a multimodal imaging approach combining histopathology with mass spectrometry imaging (MSI) and same section imaging mass cytometry (IMC) to study the effects of Salmonella Typhimurium infection in the liver of a mouse model using the S. Typhimurium strains SL3261 and SL1344. This approach enables correlation of tissue morphology and specific cell phenotypes with molecular images of tissue metabolism. IMC revealed a marked increase in immune cell markers and localization in immune aggregates in infected tissues. A correlative computational method (network analysis) was deployed to find metabolic features associated with infection and revealed metabolic clusters of acetyl carnitines, as well as phosphatidylcholine and phosphatidylethanolamine plasmalogen species, which could be associated with pro-inflammatory immune cell types. By developing an IMC marker for the detection of Salmonella LPS, we were further able to identify and characterize those cell types which contained S. Typhimurium. [dataset] Nicole Strittmatter. Holistic Characterization of a Salmonella Typhimurium Infection Model Using Integrated Molecular Imaging, metabolights_dataset, V1; 2022. https://www.ebi.ac.uk/metabolights/MTBLS2671.

284.2637 [M+K]+
PPM:11.4
Homo sapiens colorectal adenocarcinoma DESI ()
80TopL, 50TopR, 70BottomL, 60BottomR-profile - MTBLS415
Resolution: 17μm, 137x136

Description

The human colorectal adenocarcinoma sample was excised during a surgical operation performed at the Imperial College Healthcare NHS Trust. The sample and procedures were carried out in accordance with ethical approval (14/EE/0024).

284.2637 [M+K]+
PPM:11.4
Homo sapiens colorectal adenocarcinoma DESI ()
520TopL, 490TopR, 510BottomL, 500BottomR-profile - MTBLS415
Resolution: 17μm, 147x131

Description

The human colorectal adenocarcinoma sample was excised during a surgical operation performed at the Imperial College Healthcare NHS Trust. The sample and procedures were carried out in accordance with ethical approval (14/EE/0024).

284.2613 [M+K]+
PPM:3
Homo sapiens colorectal adenocarcinoma DESI ()
439TopL, 409TopR, 429BottomL, 419BottomR-profile - MTBLS415
Resolution: 17μm, 157x136

Description

The human colorectal adenocarcinoma sample was excised during a surgical operation performed at the Imperial College Healthcare NHS Trust. The sample and procedures were carried out in accordance with ethical approval (14/EE/0024).

284.2637 [M+K]+
PPM:11.4
Homo sapiens NA DESI ()
160TopL,130TopR,150BottomL,140BottomR-profile - MTBLS415
Resolution: 17μm, 142x136

Description

210.1495 [M+H-2H2O]+
PPM:3.1
Homo sapiens esophagus DESI ()
LNTO29_16_3 - MTBLS385
Resolution: 17μm, 108x107

Description

284.2576 [M+K]+
PPM:10
Homo sapiens colorectal adenocarcinoma DESI ()
240TopL, 210TopR, 230BottomL, 220BottomR-centroid - MTBLS176
Resolution: 50μm, 142x141

Description


Isovalerylcarnitine is the phenotypic abnormality in isovaleric acidemia (OMIM 243500) resulting from an accumulation of isovaleric acid, which is toxic to the central nervous system. Isovaleric acidemia is an autosomal recessive inborn error of leucine metabolism caused by a deficiency of the mitochondrial enzyme isovaleryl-CoA dehydrogenase (EC 1.3.99.10) resulting in the accumulation of derivatives of isovaleryl-CoA. It was the first organic acidemia recognized in humans and can cause significant morbidity and mortality. Early diagnosis and treatment with a protein restricted diet and supplementation with carnitine and glycine are effective in promoting normal development in severely affected individuals. An alternative pathway through glycine-N-acylase (EC 2.3.1.13) allows detoxification by producing isovalerylglycine, which is excreted. Thus, isovalerylcarnitine and isovalerylglycine are the hallmarks of this disorder in plasma and urine, respectively, and are elevated regardless of a patients metabolic condition (PMID: 16602101). Moreover, isovalerylcarnitine is found to be associated with celiac disease and very long-chain acyl-CoA dehydrogenase deficiency (VLCAD), which are also inborn errors of metabolism. Isovalerylcarnitine is the phenotypic abnormality in isovaleric acidemia (OMIM 243500) resulting from an accumulation of isovaleric acid, which is toxic to the central nervous system. Isovaleric acidemia is an autosomal recessive inborn error of leucine metabolism caused by a deficiency of the mitochondrial enzyme isovaleryl-CoA dehydrogenase (EC 1.3.99.10) resulting in the accumulation of derivatives of isovaleryl-CoA. It was the first organic acidemia recognized in humans and can cause significant morbidity and mortality. Early diagnosis and treatment with a protein restricted diet and supplementation with carnitine and glycine are effective in promoting normal development in severely affected individuals. An alternative pathway through glycine-N-acylase (EC 2.3.1.13) allows detoxification by producing isovalerylglycine, which is excreted. Thus, isovalerylcarnitine and isovalerylglycine are the hallmarks of this disorder in plasma and urine, respectively, and are elevated regardless of a patients metabolic condition. (PMID: 16602101) [HMDB] Isovalerylcarnitine is a product of the catabolism of L-leucine. Isovalerylcarnitine is also a selective and reversible calpain activator that induces apoptosis[1][2][3].