Sign in or Register   Sign in or Register
  |  

Mouse Anti-PNPLA6 Recombinant Antibody (3D10) (CBMAB-P2277-YC)

Provided herein is a Mouse monoclonal antibody against Human Patatin Like Phospholipase Domain Containing 6. The antibody can be used for immunoassay techniques, such as ELISA, IHC-P, WB.
See all PNPLA6 antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
3D10
Antibody Isotype
IgG2a, κ
Application
ELISA, IHC-P, WB

Basic Information

Immunogen
NTE (NP_006693.2, 1229-1327 aa) partial recombinant protein with GST tag. Immunogen sequence: ESRRADVLAF PSSGFTDLAE IVSRIEPPTS YVSDGCADGE ESDCLTEYEE DAGPDCSRDE GGSPEGASPS TASEMEEEKS ILRQRRCLPQ EPPGSATDA
Specificity
Human
Antibody Isotype
IgG2a, κ
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Storage
Store at 4°C short term (1-2 weeks). Aliquot and store at-20°C long term. Avoid repeated freeze/thaw cycles.
Epitope
aa 1229-1327

Target

Full Name
Patatin Like Phospholipase Domain Containing 6
Introduction
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.
Entrez Gene ID
10908
UniProt ID
Q8IY17
Alternative Names
BNHS; SPG39; LNMS; NTEMND; iPLA2delta; sws; NTE; OMCS
Function
Phospholipase B that deacylates intracellular phosphatidylcholine (PtdCho), generating glycerophosphocholine (GroPtdCho). This deacylation occurs at both sn-2 and sn-1 positions of PtdCho. Catalyzes the hydrolysis of several naturally occurring membrane-associated lipids (PubMed:11927584).
Hydrolyzes lysophospholipids and monoacylglycerols, preferring the 1-acyl to the 2-acyl isomer. Does not catalyze hydrolysis of di- or triacylglycerols or fatty acid amides (PubMed:11927584).
Biological Process
Glycerophospholipid catabolic processTAS:Reactome
Phosphatidylcholine metabolic processIEA:InterPro
Cellular Location
Endoplasmic reticulum membrane
Involvement in disease
Spastic paraplegia 39, autosomal recessive (SPG39):
A form of spastic paraplegia, 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.
Boucher-Neuhauser syndrome (BNHS):
An autosomal recessive disorder characterized by spinocerebellar ataxia, hypogonadotropic hypogonadism, and visual impairment due to chorioretinal dystrophy. The age at onset is variable, but most patients develop 1 or more symptoms in the first decade of life. Chorioretinal dystrophy may not always be present.
Laurence-Moon syndrome (LNMS):
An autosomal recessive syndrome characterized by progressive spinocerebellar degeneration, spastic paraplegia, intellectual disability, hypogonadism, dwarfism, and chorioretinopathy. Trichomegaly is absent.
Oliver-McFarlane syndrome (OMCS):
A rare autosomal recessive, congenital syndrome characterized by trichomegaly, severe chorioretinal atrophy and multiple pituitary hormone deficiencies. It results in intellectual impairment and dwarfism, if untreated. Clinical features include hypogonadotropic hypogonadism during puberty, pigmentary retinal degeneration, ataxia, spastic paraplegia, and peripheral neuropathy.
Topology
Lumenal: 1-59
Helical: 60-80
Cytoplasmic: 81-1375
PTM
Glycosylated.
Ask a question We look forward to hearing from you.
0 reviews or Q&As

Purchasing FAQs
Technical FAQs

Loading...
Have you used Mouse Anti-PNPLA6 Recombinant Antibody (3D10)?
Submit a review and get a Coupon or an Amazon gift card. 20% off Coupon $30 eGift Card
Submit a review
Loading...
For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

Online Inquiry

Documents

Contact us

  • Tel: (USA)
  • (UK)
  • Fax:
  • Email:

Submit A Review

Go to
Compare