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Huntingtin Antibody (PA1-004) in ICC/IF
Immunofluorescent analysis of Caspase cleaved Htt (green) in 293T cells transfected with Htt23Q and Htt148Q stop constructs ending in amino acid 586. Formalin fixed cells were permeabilized with 0.1% Triton X-100 in TBS for 10 minutes at room temperature and blocked with 5% normal goat serum for 15 minutes at room temperature. Cells were probed with an Htt neo-epitope 586 polyclonal antibody (Product # PA1-... View More
Product Details
PA1-004
Applications
Tested Dilution
Publications
Product Specifications
Species Reactivity
Human,
Mouse
Published species
Human
Host/Isotype
Rabbit
/ IgG
Class
Polyclonal
Type
Antibody
Immunogen
Synthetic peptide conjugated to KLH via cysteine corresponding to CPSDSSEIVLD (577-586) of Human HTT.
Conjugate
Form
Liquid
Concentration
0.6 mg/mL
Purification
Affinity chromatography
Storage buffer
PBS with 1mg/mL BSA
Contains
0.05% sodium azide
Storage conditions
-20°C
Shipping conditions
Ambient (domestic); Wet ice (international)
RRID
AB_2539851
Product Specific Information
Neoepitope antibodies distinguish smaller cleaved fragments or processed forms of proteins versus the intact full-length or precursor by using a designed peptide purification process to maximize immunoreactivity to a specific cleavage site.
Human HTT caspase cleavage sites generate fragment-specific forms of the protein. Caspase-3/7 has been shown to generate cleavage sites at animo acids 513 and 552. Caspase-2 cleaves at amino acid 552 and caspase-6 at amino acid 586. Neo-specific antibody PA1-004 recognizes the 586 cleaved fragment without detecting the full-length form.
Target Information
Huntingtin is a disease gene linked to Huntington's disease (HD), a neurodegenerative disorder characterized by loss of striatal neurons. This is thought to be caused by an expanded, unstable trinucleotide repeat in the huntingtin gene, which translates as a polyglutamine repeat in the protein product. HD is a mid-life onset autosomal dominant neurodegenerative disease that is characterized by psychiatric disorders, dementia, and involuntary movements (chorea), leading to death in 10-20 years.The huntingtin locus is large, spanning 180 kb and consisting of 67 exons. The huntingtin gene is widely expressed and is required for normal development. It is expressed as 2 alternatively polyadenylated forms displaying different relative abundance in various fetal and adult tissues. The larger transcript is approximately 13.7 kb and is expressed predominantly in adult and fetal brain whereas the smaller transcript of approximately 10.3 kb is more widely expressed. The genetic defect leading to Huntington's disease may not necessarily eliminate transcription, but may confer a new property on the mRNA or alter the function of the protein.
For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.
Bioinformatics
Protein Aliases: HD protein; HD protein homolog; Huntingtin; Huntington disease gene homolog; Huntington disease protein; huntington disease protein homolog
Gene Aliases: AI256365; C430023I11Rik; HD; Hdh; HTT; IT15
UniProt ID: (Human) P42858
Entrez Gene ID: (Human) 3064, (Mouse) 15194
establishment of mitotic spindle orientation
retrograde vesicle-mediated transport, Golgi to ER
apoptotic process
Golgi organization
central nervous system development
positive regulation of inositol 1,4,5-trisphosphate-sensitive calcium-release channel activity
regulation of protein phosphatase type 2A activity
vocal learning
positive regulation of cilium assembly
vesicle transport along microtubule
animal organ development
mRNA transport
negative regulation of cysteine-type endopeptidase activity
negative regulation of extrinsic apoptotic signaling pathway
urea cycle
citrulline metabolic process
protein import into nucleus
mitochondrial transport
ER to Golgi vesicle-mediated transport
mitochondrion organization
endoplasmic reticulum organization
dopamine receptor signaling pathway
spermatogenesis
gastrulation
brain development
cell aging
learning or memory
learning
grooming behavior
locomotory behavior
axo-dendritic transport
associative learning
determination of adult lifespan
visual learning
anatomical structure morphogenesis
embryo development
anterior/posterior pattern specification
endosomal transport
lactate biosynthetic process from pyruvate
quinolinate biosynthetic process
striatum development
olfactory lobe development
neural plate formation
neurogenesis
peptide hormone secretion
insulin secretion
social behavior
hormone metabolic process
negative regulation of apoptotic process
negative regulation of neuron apoptotic process
regulation of mitochondrial membrane permeability
regulation of synaptic plasticity
paraxial mesoderm formation
neuron development
neuron apoptotic process
response to calcium ion
regulation of mitochondrial membrane potential
L-glutamate import
iron ion homeostasis
negative regulation of neuron death
Performance Guarantee
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