Ms X Huntingtin Protein, a.a. 181-810, clone 1HU-4C8 (Monoclonal Antibody)

Species Reactivity	Key Applications	Host	Format	Antibody Type
H, M, R, Mk, Ht, Rb	ELISA, ICC, IH(P), IP, WB	M	Ascites	Monoclonal Antibody

Species Reactivity

Key Applications

Host

Format

Antibody Type

H, M, R, Mk, Ht, Rb

ELISA, ICC, IH(P), IP, WB

Ascites

Monoclonal Antibody

Description
Catalogue Number	MAB2166
Brand Family	Chemicon®
Trade Name	Chemicon
Description	Anti-Huntingtin Protein Antibody, a.a. 181-810, clone 1HU-4C8
Alternate Names	Huntingtin Huntington's Disease Protein HD Protein
Background Information	Huntington disease (HD) is a hereditary, progressive, neurodegenerative ailment characterized by personality changes, motor impairment and subcortical dementia. The molecular basis of the disease involves the expansion of the trinucleotide CAG, coding for polyglutamine in the first exon of a chromosome four gene (4p16.3), which normally produces a widely expressed 3136 a.a. (~350 kDa) protein huntingtin with unclear function. The protein is found in the perinuclear region along with microtubules, and in the centrosomal region along with gamma-tubulin. Huntingtin is necessary for neuronal survival and is involved in synaptic vesicle trafficking, microtubule binding and may also have a role in apoptosis. In the HD condition, neuronal cells with the mutant form of huntingtin possess intranuclear aggregations of the N-terminal fragment, causing damaging inclusions in perinuclear locations and striatal neuron cell death. Wild-type huntington and anti-huntingtin reduce aggregation and cellular toxicity of the mutant huntingtin form in mammalian cell models of HD. Huntingtin is known to interact with GAPDH, HAP-1, SP1 and TAFII130.

Description

Catalogue Number

MAB2166

Brand Family

Chemicon®

Trade Name

Chemicon

Description

Anti-Huntingtin Protein Antibody, a.a. 181-810, clone 1HU-4C8

Alternate Names

Huntingtin
Huntington's Disease Protein
HD Protein

Background Information

Huntington disease (HD) is a hereditary, progressive, neurodegenerative ailment characterized by personality changes, motor impairment and subcortical dementia. The molecular basis of the disease involves the expansion of the trinucleotide CAG, coding for polyglutamine in the first exon of a chromosome four gene (4p16.3), which normally produces a widely expressed 3136 a.a. (~350 kDa) protein huntingtin with unclear function. The protein is found in the perinuclear region along with microtubules, and in the centrosomal region along with gamma-tubulin. Huntingtin is necessary for neuronal survival and is involved in synaptic vesicle trafficking, microtubule binding and may also have a role in apoptosis. In the HD condition, neuronal cells with the mutant form of huntingtin possess intranuclear aggregations of the N-terminal fragment, causing damaging inclusions in perinuclear locations and striatal neuron cell death. Wild-type huntington and anti-huntingtin reduce aggregation and cellular toxicity of the mutant huntingtin form in mammalian cell models of HD. Huntingtin is known to interact with GAPDH, HAP-1, SP1 and TAFII130.

Product Information
Format	Ascites
Control	Normal human cerebral cortex lysate
Presentation	Ascites mouse monoclonal IgG1κ liquid containing no preservative

Product Information

Format

Ascites

Control

Normal human cerebral cortex lysate

Presentation

Ascites mouse monoclonal IgG1κ liquid containing no preservative

Applications
Application	Anti-Huntingtin Protein Antibody, a.a. 181-810, clone 1HU-4C8 is an antibody against Huntingtin Protein for use in ELISA, IC, IH(P), IP & WB.
Key Applications	ELISA Immunocytochemistry Immunohistochemistry (Paraffin) Immunoprecipitation Western Blotting
Application Notes	ELISA: A 1:500-1:5,000 dilution of a previous lot was used on ELISA. Immunohistochemistry: A 1:500-1:5,000 dilution from a previous lot was used on frozen and microwave oven treated paraffin sections (human tissue). Immunocytochemistry: 1:500-1:5,000 on a previous lot was used on transfected cells. Immunoprecipitation: A 1:500-1:5,000 dilution of a previous lot was used on immunoprecipitation. Western blot: 1:500-1:5,000. Should detect a band migrating at approximately 350-400 kDa by Western blot (Nature Genetics 10:104-110.). Optimal working dilutions must be determined by the end user.

Applications

Application

Anti-Huntingtin Protein Antibody, a.a. 181-810, clone 1HU-4C8 is an antibody against Huntingtin Protein for use in ELISA, IC, IH(P), IP & WB.

Key Applications

ELISA
Immunocytochemistry
Immunohistochemistry (Paraffin)
Immunoprecipitation
Western Blotting

Application Notes

ELISA:
A 1:500-1:5,000 dilution of a previous lot was used on ELISA.

Immunohistochemistry:
A 1:500-1:5,000 dilution from a previous lot was used on frozen and microwave oven treated paraffin sections (human tissue).

Immunocytochemistry:
1:500-1:5,000 on a previous lot was used on transfected cells.

Immunoprecipitation:
A 1:500-1:5,000 dilution of a previous lot was used on immunoprecipitation.

Western blot:
1:500-1:5,000. Should detect a band migrating at approximately 350-400 kDa by Western blot (Nature Genetics 10:104-110.).

Optimal working dilutions must be determined by the end user.

Biological Information
Immunogen	Huntingtin fragment from a.a. 181 to 810 as a fusion protein.
Epitope	a.a. 181-810
Clone	1HU-4C8
Concentration	Please refer to the Certificate of Analysis for the lot-specific concentration.
Host	Mouse
Specificity	Huntingtin Protein. No detectable cross reactivity to other proteins by Western blot.
Isotype	IgG1κ
Species Reactivity	Human Mouse Rat Monkey Hamster Rabbit
Species Reactivity Note	Rat. Expected to react with human, monkey, hamster and mouse based on sequence homology
Antibody Type	Monoclonal Antibody
Entrez Gene Number	NM_002111.6
Entrez Gene Summary	Huntingtin is a disease gene linked to Huntington's disease, 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. A fairly broad range in the number of trinucleotide repeats has been identified in normal controls, and repeat numbers in excess of 40 have been described as pathological. 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. One candidate is the huntingtin-associated protein-1, highly expressed in brain, which has increased affinity for huntingtin protein with expanded polyglutamine repeats. This gene contains an upstream open reading frame in the 5' UTR that inhibits expression of the huntingtin gene product through translational repression
Gene Symbol	HTT IT15 SLC6A4 OCD1 HD
Purification Method	Unpurified
UniProt Number	P42858
UniProt Summary	FUNCTION: SwissProt: P42858 # May play a role in microtubule-mediated transport or vesicle function. SIZE: 3144 amino acids; 347860 Da SUBUNIT: Binds SH3GLB1 (By similarity). Interacts through its N- terminus with FNBP3. Interacts with PQBP1, SETD2 and SYVN. SUBCELLULAR LOCATION: Cytoplasm. Nucleus. TISSUE SPECIFICITY: Widely expressed with the highest level of expression in the brain (nerve fibers, varicosities, and nerve endings). In the brain, the regions where it can be mainly found are the cerebellar cortex, the neocortex, the striatum, and the hippocampal formation. PTM: Cleaved by apopain downstream of the polyglutamine stretch. The resulting amino-terminal fragment is cytotoxic and provokes apoptosis. & Forms with expanded polyglutamine expansion are specifically ubiquitinated by SYVN1, which promotes their proteasomal degradation. DISEASE: SwissProt: P42858 # Defects in HD are the cause of Huntington disease (HD) [MIM:143100]. HD is an autosomal dominant neurodegenerative disorder characterized by involuntary movements (chorea), general motor impairment, psychiatric disorders and dementia. Onset of the disease occurs usually in the third or fourth decade of life and symptoms progressively worsen leading to death in 10 to 20 years. Onset and clinical course depend on the degree of poly-Gln repeat expansion, longer expansions resulting in earlier onset and more severe clinical manifestations. HD affects 1 in 10,000 individuals of European origin. Neuropathology of Huntington disease displays a distinctive pattern with loss of neurons, especially in the caudate and putamen (striatum). SIMILARITY: SwissProt: P42858 ## Belongs to the hungtintin family. & Contains 10 HEAT repeats.
Molecular Weight	~ 350-400 kDa

Biological Information

Immunogen

Huntingtin fragment from a.a. 181 to 810 as a fusion protein.

Epitope

a.a. 181-810

Clone

1HU-4C8

Concentration

Please refer to the Certificate of Analysis for the lot-specific concentration.

Host

Mouse

Specificity

Huntingtin Protein. No detectable cross reactivity to other proteins by Western blot.

Isotype

IgG1κ

Species Reactivity

Human
Mouse
Rat
Monkey
Hamster
Rabbit

Species Reactivity Note

Rat. Expected to react with human, monkey, hamster and mouse based on sequence homology

Antibody Type

Monoclonal Antibody

Entrez Gene Number

NM_002111.6

Entrez Gene Summary

Huntingtin is a disease gene linked to Huntington's disease, 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. A fairly broad range in the number of trinucleotide repeats has been identified in normal controls, and repeat numbers in excess of 40 have been described as pathological. 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. One candidate is the huntingtin-associated protein-1, highly expressed in brain, which has increased affinity for huntingtin protein with expanded polyglutamine repeats. This gene contains an upstream open reading frame in the 5' UTR that inhibits expression of the huntingtin gene product through translational repression

Gene Symbol

HTT
IT15
SLC6A4
OCD1
HD

Purification Method

Unpurified

UniProt Number

P42858

UniProt Summary

FUNCTION: SwissProt: P42858 # May play a role in microtubule-mediated transport or vesicle function.
SIZE: 3144 amino acids; 347860 Da
SUBUNIT: Binds SH3GLB1 (By similarity). Interacts through its N- terminus with FNBP3. Interacts with PQBP1, SETD2 and SYVN.
SUBCELLULAR LOCATION: Cytoplasm. Nucleus.
TISSUE SPECIFICITY: Widely expressed with the highest level of expression in the brain (nerve fibers, varicosities, and nerve endings). In the brain, the regions where it can be mainly found are the cerebellar cortex, the neocortex, the striatum, and the hippocampal formation.
PTM: Cleaved by apopain downstream of the polyglutamine stretch. The resulting amino-terminal fragment is cytotoxic and provokes apoptosis. & Forms with expanded polyglutamine expansion are specifically ubiquitinated by SYVN1, which promotes their proteasomal degradation.
DISEASE: SwissProt: P42858 # Defects in HD are the cause of Huntington disease (HD) [MIM:143100]. HD is an autosomal dominant neurodegenerative disorder characterized by involuntary movements (chorea), general motor impairment, psychiatric disorders and dementia. Onset of the disease occurs usually in the third or fourth decade of life and symptoms progressively worsen leading to death in 10 to 20 years. Onset and clinical course depend on the degree of poly-Gln repeat expansion, longer expansions resulting in earlier onset and more severe clinical manifestations. HD affects 1 in 10,000 individuals of European origin. Neuropathology of Huntington disease displays a distinctive pattern with loss of neurons, especially in the caudate and putamen (striatum).
SIMILARITY: SwissProt: P42858 ## Belongs to the hungtintin family. & Contains 10 HEAT repeats.

Molecular Weight

~ 350-400 kDa

Product Usage Statements
Quality Assurance	Routinely evaluated by Western Blot on rat brain lysates. Western Blot Analysis: 1:1000 dilution of this lot detected huntingtin protein on 10 μg of rat brain lysates.
Usage Statement	Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.

Product Usage Statements

Quality Assurance

Routinely evaluated by Western Blot on rat brain lysates.

Western Blot Analysis:
1:1000 dilution of this lot detected huntingtin protein on 10 μg of rat brain lysates.

Usage Statement

Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.

Storage and Shipping Information
Storage Conditions	Stable for 1 year at -20ºC in undiluted aliquots from date of receipt. Handling Recommendations: Upon receipt, and prior to removing the cap, centrifuge the vial and gently mix the solution. Aliquot into microcentrifuge tubes and store at -20°C. Avoid repeated freeze/thaw cycles, which may damage IgG and affect product performance.

Storage and Shipping Information

Storage Conditions

Stable for 1 year at -20ºC in undiluted aliquots from date of receipt.
Handling Recommendations: Upon receipt, and prior to removing the cap, centrifuge the vial and gently mix the solution. Aliquot into microcentrifuge tubes and store at -20°C. Avoid repeated freeze/thaw cycles, which may damage IgG and affect product performance.

Packaging Information
Material Size	100 µL

Packaging Information

Material Size

100 µL

实验耗材

分子生物学试剂

蛋白与免疫学

生化试剂

细胞及检测试剂

色谱及层析

仪器设备

实验服务

办公用品

Ms X Huntingtin Protein, a.a. 181-810, clone 1HU-4C8 (Monoclonal Antibody)

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