PANK2 Antibody, Biotin Conjugated
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Catalog number
bs-8338R-Biotin
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Price
Please ask
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Size
0.1ml
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Long name
PANK2 Polyclonal Antibody, Biotin Conjugated
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Category
Conjugated Primary Antibodies
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Conjugation
Biotin
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Host Organism
Rabbit (Oryctolagus cuniculus)
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Target Antigen
PANK2
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Specificity
This is a highly specific antibody against PANK2.
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Modification
Unmodified
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Modification site
None
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Clonality
Polyclonal
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Clone
Polyclonal antibody
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Concentration
1ug per 1ul
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Source
KLH conjugated synthetic peptide derived from human PANK2
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Gene ID number
80025
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Tested applications
WB, IHC-P
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Recommended dilutions
WB(1:100-1000), IHC-P(1:100-500)
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Crossreactivity
Human, Mouse, Rat
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Crossreactive species details
Due to limited amount of testing and knowledge, not every possible cross-reactivity is known.
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Antigen background
Defects in PANK2 are the cause of neurodegeneration with brain iron accumulation type 1 (NBIA1); also known as pantothenate kinase-associated neurodegeneration (PKAN) or Hallervorden-Spatz syndrome (HSS). It is an autosomal recessive neurodegenerative disorder associated with iron accumulation in the brain, primarily in the basal ganglia. Clinical manifestations include progressive muscle spasticity, hyperreflexia, muscle rigidity, dystonia, dysarthria, and intellectual deterioration which progresses to severe dementia over several years. It is clinically classified into classic, atypical, and intermediate phenotypes. Classic forms present with onset in the first decade, rapid progression, loss of independent ambulation within 15 years. Atypical forms have onset in the second decade, slow progression, maintenance of independent ambulation up to 40 years later. Intermediate forms manifest onset in the first decade with slow progression or onset in the second decade with rapid progression. Patients with early onset tend to also develop pigmentary retinopathy, whereas those with later onset tend to also have speech disorders and psychiatric features. All patients have the 'eye of the tiger' sign on brain MRI.Defects in PANK2 are the cause of hypoprebetalipoproteinemia, acanthocytosis, retinitis pigmentosa, and pallidal degeneration (HARP). HARP is a rare syndrome with many clinical similarities to NBIA1.
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Purification method
This antibody was purified via Protein A.
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Storage conditions
Keep the antibody in an aqueous buffered solution containing 1% BSA, 50% glycerol and 0.09% sodium azide. Store refrigerated at 2 to 8 degrees Celcius for up to 1 year.
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Excitation Emission
N/A
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Synonyms
C20orf48; HARP; hPANK2; HSS; MGC15053; NBIA1; PANK2; PANK2_HUMAN; Pantothenate kinase 2 Hallervorden Spatz syndrome; Pantothenate kinase 2; Pantothenic acid kinase 2; PKAN; RP23 387C21.4.
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Properties
If you buy Antibodies supplied by Bioss Primary Conjugated Antibodies they should be stored frozen at - 24°C for long term storage and for short term at + 5°C. Biotin conjugates can be detected by horseradish peroxidase, alkaline phosphatase substrates or anti biotin conjugated antibodies. Avidin and Streptavidin bind to the small biotin and are couple to HRP or AP for ELISA. To break the streptavidin Biotin bond we suggest to use a 6 molar guanidine HCl solution with acidity of pH 1.6.
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Additional conjugation
Biotinylated
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French translation
anticorps
Gene info
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Identity
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Gene
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Long gene name
PANK2 antisense RNA 1
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Locus
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Discovery year
2019-09-19
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Entrez gene record
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Classification
Gene info
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Identity
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Gene
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Long gene name
pantothenate kinase 2
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Synonyms gene
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Synonyms gene name
- neurodegeneration with brain iron accumulation 1 (Hallervorden-Spatz syndrome)
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Synonyms
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Synonyms name
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GenBank acession
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Locus
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Discovery year
2001-06-21
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Entrez gene record
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Pubmed identfication
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RefSeq identity
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Classification
- Pantothenate kinases
- MicroRNA protein coding host genes
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VEGA ID
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Locus Specific Databases
MeSH Data
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Name
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Concept
Scope note:
Identification of proteins or peptides that have been electrophoretically separated by blot transferring from the electrophoresis gel to strips of nitrocellulose paper, followed by labeling with antibody probes.
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Tree numbers
- E05.196.401.143
- E05.301.300.096
- E05.478.566.320.200
- E05.601.262
- E05.601.470.320.200
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Qualifiers
ethics, trends, veterinary, history, classification, economics, instrumentation, methods, standards, statistics & numerical data
Product images
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