Palmitoyl-Protein Thioesterase 1 (PPT1) Kits ELISA

The protein encoded by PPT1 is a small glycoprotein involved in the catabolism of lipid-modified proteins during lysosomal degradation. De plus, nous expédions PPT1 Anticorps (105) et PPT1 Protéines (14) et beaucoup plus de produits pour cette protéine.

list all ELISA KIts Gène GeneID UniProt
PPT1 5538 P50897
PPT1 19063 O88531
PPT1 29411 P45479
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Catalogue No. Reactivité Sensibilité Gamme Images Quantité Fournisseur Livraison Prix Détails
Souris 7 pg/mL 28-1800 pg/mL Typical standard curve 96 Tests Connectez-vous pour afficher 15 to 18 Days
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Plus Kits ELISA pour PPT1 partenaires d'interaction

Human Palmitoyl-Protein Thioesterase 1 (PPT1) interaction partners

  1. Targeting PPT1 blocks mTOR signaling in a manner distinct from catalytic inhibitors, while concurrently inhibiting autophagy, thereby providing a new strategy for cancer therapy.

  2. the combination of elevated glycolysis and deficient MRPL13 activity was closely linked to CLN1-mediated tumor activity in human hepatoma cells

  3. Proteomics analysis on isolated cilia revealed 660 proteins, which differed in their abundance levels between wild type and Ppt1 knock out.

  4. we reveal the existence of a positive feedback loop, where palmitoylation of PPT1 results in decreased activity and subsequent elevation in the amount of palmitoylated proteins.

  5. analysis of the palmitoyl protein thioesterase 1 interactome in SH-SY5Y human neuroblastoma cells

  6. Data (including data from knockout mice) suggest that deficiency of PPT1 leads to accumulation of granular osmiophilic deposits in many cell types, especially in astrocytes. [review-like article]

  7. Data suggest that human monocytes and macrophages express PPT1; PPT1 appears to contribute 32-40% of 2-arachidonylglycerol hydrolysis activity in THP1 monocyte cell line.

  8. This neuroimaging finding in PPT1-related neuronal ceroid lipofuscinosis was not previously reported.

  9. Stop codon read-through with PTC124 induces palmitoyl-protein thioesterase-1 activity, reduces thioester load and suppresses apoptosis in cultured cells from Infantile neuronal ceroid lipofuscinosis patients.

  10. Results describe the correlation between the three-dimensional structural changes in mutant palmitoyl protein thioesterase 1 and biochemical phenotypes.

  11. mutated in neuronal ceroid lipofuscinosis

  12. The clinical, biochemical, and molecular genetic aspects of lysosomal storage disorders are discussed in this review

  13. The crystal structure of palmitoyl protein thioesterase-2 (PPT2) reveals the basis for divergent substrate specificities of the two lysosomal thioesterases, PPT1 and PPT2.

  14. there is a close correlation between CLN2 and CLN1 expression and colorectal carcinoma progression and metastasis and suggest that they may be potential molecular targets

  15. Results show that PPT1-deficiency causes a defect in fluid-phase and receptor-mediated endocytosis.

  16. ER stress due to PPT1-deficiency increases ROS and disrupts calcium homeostasis activating caspase-9 and caspase-9 activation mediates caspase-3 activation and apoptosis contributing to rapid neurodegeneration in INCL.

  17. Adult neuronal ceroid lipofuscinosis caused by deficiency in palmitoyl protein thioesterase 1.

  18. Palmitoyl protein thioesterase-1 deficiency impairs synaptic vesicle recycling at nerve terminals in humans and mice

  19. study presents clinical and diagnostic investigations in six children with variant late infantile neuronal ceroid lipofuscinosis and mutations in CLN1 including a new large-scale deletion on one allele

  20. Using shotgun mass spectrometry, we found this protein differentially expressed in the dorsolateral prefrontal cortex from patients with schizophrenia.

Mouse (Murine) Palmitoyl-Protein Thioesterase 1 (PPT1) interaction partners

  1. Our results indicate that PPT1 deficiency causes alterations in the mitochondrial respiratory chain.

  2. Proteomics analysis on isolated cilia revealed 660 proteins, which differed in their abundance levels between wild type and Ppt1 knock out.

  3. In the novel Cln1(R151X) mouse model of INCL.

  4. Parkinson-like motor/sensorimotor deficits in Cln1-/- mice are not mediated by dopamine deficiency.

  5. Data show that Cln1 mutations disrupt the maturation of cathepsin D in lysosome contributing to neuropathology of infantile neuronal ceroid lipofuscinoses and suggest cathepsin D deficiency to be common link between infantile and congenital form.

  6. The authors have generated a Cln1 R151X point mutation mouse model that recapitulates the molecular, neuropathological and behavioral phenotypes of neuronal ceroid lipofuscinoses.

  7. Data (including data from knockout mice) suggest that deficiency of Ppt1 leads to accumulation of granular osmiophilic deposits in many cell types, especially in astrocytes. [review-like article]

  8. Data suggest that mouse macrophages express PPT1; PPT1 appears to contribute to 2-arachidonylglycerol hydrolysis activity in peritoneal macrophages in culture.

  9. The simultaneous loss of both Cln1 and Cln5 genes might enhance the typical pathological phenotypes of these mice by disrupting or downregulating shared or convergent pathogenic pathways.

  10. These results uncover a previously unknown phenotype associated with PPT1 deficiency, that of altered thermoregulation, which is associated with impaired lipolysis and neurotransmitter release to brown adipose tissue during cold exposure.

  11. The addition of the small molecule PPT1 mimetic can further increase that response.

  12. Glutamate receptor 4 (GluR4) AMPA receptor hypofunction and NMDA receptor hyperfunction phenotype show increased sensitivity in Ppt1-deficient neurons.

  13. Data show a progressive breakdown of axons and synapses in the brains of two different models of NCL: Ppt1(-/-) model of infantile NCL and Cln6(nclf) model of variant late-infantile NCL.

  14. The polarized axonal targeting of PPT1 may well indicate a role for PPT1 in the exocytotic pathway of neurons.

  15. was only detected in developing testis not ovary

  16. The severely affected PPT1-/- mouse CNS exhibited reduced volume of both cortical and subcortical regions, but with sparing of the cerebellum

  17. Autopsy of PPT1 mutant mice revealed a severe loss of brain mass and accumulation of autofluorescent granular osmiophilic deposits, both characteristic of Neuronal Ceroid Lipofuscinosis.

  18. We conclude that from an early age, neurons deficient in PPT1 enzyme activity display intrinsically abnormal properties that could potentially explain key features of the clinical disease, such as myoclonus and seizures.

  19. Palmitoyl-protein thioesterase-1 deficiency mediates the activation of the unfolded protein response and neuronal apoptosis in infantile neuronal ceroid lipofuscinosis.

  20. Age-dependent increase in LPC levels in the PPT1-KO mouse brain positively correlates with elevated expression of the genes characteristically associated with phagocytes.

PPT1 profil antigène

Antigen Summary

The protein encoded by this gene is a small glycoprotein involved in the catabolism of lipid-modified proteins during lysosomal degradation. The encoded enzyme removes thioester-linked fatty acyl groups such as palmitate from cysteine residues. Defects in this gene are a cause of infantile neuronal ceroid lipofuscinosis 1 (CLN1, or INCL) and neuronal ceroid lipofuscinosis 4 (CLN4). Two transcript variants encoding different isoforms have been found for this gene.

Gene names and symbols associated with PPT1

  • palmitoyl-protein thioesterase 1 (PPT1) anticorps
  • palmitoyl-protein thioesterase 1 (MGYG_00692) anticorps
  • palmitoyl-protein thioesterase 1 (Ppt1) anticorps
  • Palmitoyl-protein thioesterase 1 (ppt-1) anticorps
  • palmitoyl-protein thioesterase 1 (ceroid-lipofuscinosis, neuronal 1, infantile) (ppt1) anticorps
  • palmitoyl-protein thioesterase 1 L homeolog (ppt1.L) anticorps
  • 9530043G02Rik anticorps
  • AA960502 anticorps
  • C77813 anticorps
  • CLN1 anticorps
  • D4Ertd184e anticorps
  • INCL anticorps
  • Ppt anticorps
  • PPT-1 anticorps
  • ppt1 anticorps
  • wu:fj17f04 anticorps
  • zgc:63969 anticorps

Protein level used designations for PPT1

ceroid-palmitoyl-palmitoyl-protein thioesterase 1 , palmitoyl-protein hydrolase 1 , palmitoyl-protein thioesterase 1 , PPT-1 , palmitoyl-protein thioesterase 1 (ceroid-lipofuscinosis, neuronal 1, infantile) , palmitoyl protein thioesterase , Palmitoyl-protein hydrolase 1 , palmitoyl-protein thioesterase 1 L homeolog

GENE ID SPECIES
5538 Homo sapiens
10031925 Arthroderma gypseum CBS 118893
19063 Mus musculus
29411 Rattus norvegicus
419681 Gallus gallus
475316 Canis lupus familiaris
281421 Bos taurus
191744 Caenorhabditis elegans
406648 Danio rerio
100720295 Cavia porcellus
102126191 Macaca fascicularis
100037210 Xenopus laevis
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