Mouse monoclonal antibody Anti-Human HD | MyBioSource MBS120398 product information

This webpage contains legacy information. The product is either no longer available from the supplier or has been delisted at Labome.

product summary

company name :

MyBioSource

product type :

antibody

product name :

Mouse monoclonal antibody Anti-Human HD

catalog :

MBS120398

quantity :

0.1 mg

price :

345 USD

clonality :

monoclonal

host :

mouse

conjugate :

nonconjugated

clone name :

2401C1a

reactivity :

human

application :

immunocytochemistry

product information

catalog number :

MBS120398

products type :

Antibody

products full name :

Mouse monoclonal antibody Anti-Human HD

products short name :

products name syn :

Homo sapiens huntingtin (Huntington disease) (HD), mRNA.; HTT; IT15

other names :

Homo sapiens huntingtin (HTT), mRNA; Huntingtin; huntingtin; huntington disease protein; huntingtin; Huntington disease protein; HD protein

products gene name :

other gene names :

HTT; HTT; HD; IT15

uniprot entry name :

HD_HUMAN

clonality :

Monoclonal

isotype :

IgG2b

clone :

2401C1a

host :

Mouse

reactivity :

Human

sequence length :

13669

concentration :

100 ug/ml (1.0 ml)

tested application :

Dot Blot (DB), Immunocytochemistry (ICC)

other info1 :

Preparation: This antibody was purified using protein G column chromatography from culture supernatant of hybridoma cultured in a medium containing bovine IgG-depleted (approximately 95%) fetal bovine serum.

other info2 :

Sterility: Filtered through a 0.22 um membrane.

products description :

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.[NCBI Entrez Gene Summary]

ncbi gi num :

588282786

ncbi acc num :

NP_002102.4

ncbi gb acc num :

NM_002111

ncbi mol weight :

347,603 Da

ncbi pathways :

Direct P53 Effectors Pathway (137939); EGFR1 Signaling Pathway (198782); Huntington's Disease Pathway (83100); Huntington's Disease Pathway (512)

ncbi summary :

uniprot summary :

Huntingtin: may play a role in microtubule-mediated transport or vesicle function. 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. Defects are the cause of 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. 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 isoforms displaying different relative abundance in various fetal and adult tissues. Protein type: Cytoskeletal. Chromosomal Location of Human Ortholog: 4p16.3. Cellular Component: Golgi apparatus; protein complex; cytoplasmic vesicle membrane; mitochondrion; endoplasmic reticulum; dendrite; autophagic vacuole; inclusion body; cytosol; nucleoplasm; axon; late endosome; cytoplasm; nucleus. Molecular Function: identical protein binding; protein binding; p53 binding; dynein intermediate chain binding; beta-tubulin binding; diazepam binding; transcription factor binding. Biological Process: ER to Golgi vesicle-mediated transport; citrulline metabolic process; paraxial mesoderm formation; regulation of protein phosphatase type 2A activity; regulation of synaptic plasticity; locomotory behavior; determination of adult life span; endosome transport; anterior/posterior pattern formation; L-glutamate import; regulation of mitochondrial membrane potential; establishment of mitotic spindle orientation; protein import into nucleus; quinolinate biosynthetic process; organ development; vesicle transport along microtubule; retrograde vesicle-mediated transport, Golgi to ER; visual learning; negative regulation of neuron apoptosis; Golgi organization and biogenesis; endoplasmic reticulum organization and biogenesis; grooming behavior; positive regulation of inositol-1,4,5-triphosphate receptor activity; striatum development; axon cargo transport; cell aging; olfactory lobe development; social behavior; lactate biosynthetic process from pyruvate; neuron apoptosis; iron ion homeostasis; insulin secretion; dopamine receptor signaling pathway; hormone metabolic process; neuron development; spermatogenesis; regulation of mitochondrial membrane permeability; response to calcium ion; neural plate formation; urea cycle. Disease: Huntington Disease

size1 :

0.1 mg

price1 :

345 USD