Rabbit anti-Human PNPLA6 Polyclonal Antibody | anti-PNPLA6 antibody

PNPLA6, CT (PNPLA6, NTE, Neuropathy target esterase, Patatin-like phospholipase domain-containing protein 6) (FITC)

Cat. Num. AAA6335414

• Gene Names: PNPLA6; NTE; sws; SPG39; NTEMND; iPLA2delta
• Reactivity: Human
• Applications: Western Blot
• Purity: Purified by Protein A Affinity Chromatography.
• Synonyms: PNPLA6; Polyclonal Antibody; CT (PNPLA6; NTE; Neuropathy target esterase; Patatin-like phospholipase domain-containing protein 6) (FITC); Patatin-like phospholipase domain-containing protein 6; anti-PNPLA6 antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Isotype: IgG
• Purity/Purification: Purified by Protein A Affinity Chromatography.
• Form/Format: Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with Fluorescein Isothiocyanate (FITC).

• Applicable Applications for anti-PNPLA6 antibody: Western Blot (WB), FLISA
• Application Notes: WB: 1:100-500; FLISA: 1:1,000; Applications are based on unconjugated antibody.
• Immunogen: PNPLA6 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 960-990 amino acids from the C-terminal region of human PNPLA6.
• Conjugate: FITC
• Note: Preservative Free
• Special Handling: Light sensitive
• Preparation and Storage: Store product at 4 degree C if to be used immediately within two weeks. For long-term storage, aliquot to avoid repeated freezing and thawing and store at -20 degree C. Aliquots are stable at -20 degree C for 12 months after receipt. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. Caution: FITC conjugates are sensitive to light. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Western Blot (WB)

(PNPLA6 Antibody (C-term) western blot analysis in HepG2 cell line lysates (35ug/lane).This demonstrates the PNPLA6 antibody detected the PNPLA6 protein (arrow).)

Related Product Information for anti-PNPLA6 antibody

PNPLA6 is a phospholipase that deacetylates intracellular phosphatidylcholine to produce glycerophosphocholine. It is thought to function in neurite outgrowth and process elongation during neuronal differentiation. The protein is anchored to the cytoplasmic face of the endoplasmic reticulum in both neurons and non-neuronal cells. Mutations in this gene result in autosomal recessive spastic paraplegia, and the protein is the target for neurodegeneration induced by organophosphorus compounds and chemical warfare agents. Multiple transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq].

Product Categories/Family for anti-PNPLA6 antibody

Antibodies; Enzymes; Phospholipase

References

Hein, N.D., et al. Toxicol. Lett. 199(1):1-5(2010). Chen, J.X., et al. Pharmacol. Res. 62(3):259-264(2010). Greiner, A.J., et al. Biochim. Biophys. Acta 1798(8):1533-1539(2010). Hein, N.D., et al. Toxicol. Lett. 196(2):67-73(2010). Saito, A., et al. J. Hum. Genet. 54(6):317-323(2009).

NCBI and Uniprot Product Information

• NCBI GI #: 260656037
• NCBI GeneID: 10908
• NCBI Accession #: NP_001159583.1; NP_001159585.1
• NCBI GenBank Nucleotide #: NM_001166111.1
• UniProt Accession #: Q8IY17; O60859; Q86W58; Q9UG58; A6NGQ0; B4DFB9; J3KQS3
• Molecular Weight: 149,995 Da
• NCBI Official Full Name: neuropathy target esterase isoform a
• NCBI Official Synonym Full Names: patatin-like phospholipase domain containing 6
• NCBI Official Symbol: PNPLA6
• NCBI Official Synonym Symbols: NTE; sws; SPG39; NTEMND; iPLA2delta
• NCBI Protein Information: neuropathy target esterase; patatin-like phospholipase domain-containing protein 6
• UniProt Protein Name: Neuropathy target esterase
• Protein Family: Neuropathy target esterase
• UniProt Gene Name: PNPLA6
• UniProt Synonym Gene Names: NTE
• UniProt Entry Name: PLPL6_HUMAN

NCBI Description

This gene encodes a phospholipase that deacetylates intracellular phosphatidylcholine to produce glycerophosphocholine. It is thought to function in neurite outgrowth and process elongation during neuronal differentiation. The protein is anchored to the cytoplasmic face of the endoplasmic reticulum in both neurons and non-neuronal cells. Mutations in this gene result in autosomal recessive spastic paraplegia, and the protein is the target for neurodegeneration induced by organophosphorus compounds and chemical warfare agents. Multiple transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Oct 2009]

Uniprot Description

NTE: Phospholipase B that deacylates intracellular phosphatidylcholine (PtdCho), generating glycerophosphocholine (GroPtdCho). This deacylation occurs at both sn-2 and sn-1 positions of PtdCho. Its specific chemical modification by certain organophosphorus (OP) compounds leads to distal axonopathy. Defects in PNPLA6 are the cause of spastic paraplegia autosomal recessive type 39 (SPG39); also known as NTE-related motor neuron disorder (NTEMND). Spastic paraplegia is a neurodegenerative disorder characterized by a slow, gradual, progressive weakness and spasticity of the lower limbs. Rate of progression and the severity of symptoms are quite variable. Initial symptoms may include difficulty with balance, weakness and stiffness in the legs, muscle spasms, and dragging the toes when walking. In some forms of the disorder, bladder symptoms (such as incontinence) may appear, or the weakness and stiffness may spread to other parts of the body. SPG39 is associated with a motor axonopathy affecting upper and lower limbs and resulting in progressive wasting of distal upper and lower extremity muscles. Belongs to the NTE family. 3 isoforms of the human protein are produced by alternative splicing.

Protein type: Membrane protein, integral; EC 3.1.1.5; Hydrolase

Chromosomal Location of Human Ortholog: 19p13.2

Cellular Component: endoplasmic reticulum membrane; membrane; endoplasmic reticulum; integral to membrane

Molecular Function: lysophospholipase activity

Biological Process: organ morphogenesis; angiogenesis; developmental process; lipid catabolic process; phosphatidylcholine metabolic process

Disease: Laurence-moon Syndrome; Spastic Paraplegia 39, Autosomal Recessive; Oliver-mcfarlane Syndrome; Boucher-neuhauser Syndrome