Description:

    Size: 100ul

    Catalog no.: bs-13323R-A488

    Price: 380 EUR

    Product details

    Gene ID Number

    2653

    Target Antigen

    GCSH

    Modification Site

    None

    Tested applications

    IF(IHC-P)

    French translation

    anticorps

    Clonality

    Polyclonal

    Modification

    Unmodified

    Conjugation

    Alexa Fluor

    Concentration

    1ug per 1ul

    Excitation emission

    499nm/519nm

    Conjugated with

    ALEXA FLUOR® 488

    Crossreactivity

    Human, Mouse, Rat

    Clone

    Polyclonal antibody

    Recommended dilutions

    IF(IHC-P)(1:50-200)

    Purification

    Purified by Protein A.

    Category

    Conjugated Primary Antibodies

    Also known as

    Anti-GCSH PAb ALEXA FLUOR 488

    Host Organism

    Rabbit (Oryctolagus cuniculus)

    Specificity

    This is a highly specific antibody against GCSH.

    Long name

    GCSH Polyclonal Antibody, ALEXA FLUOR 488 Conjugated

    Cross-reactive species details

    Due to limited amount of testing and knowledge, not every possible cross-reactivity is known.

    Source

    This antibody was obtained by immunization of the host with KLH conjugated synthetic peptide derived from human GCSH

    Storage conditions

    Store this antibody in aqueous buffered solution containing 1% BSA, 50% glycerol and 0.09% sodium azide. Keep refrigerated at 2 to 8 degrees Celcius for up to one year.

    Synonyms

    GCE; GCSH; GCSH_HUMAN; Glycine cleavage system H protein; Glycine cleavage system H protein mitochondrial; Glycine cleavage system protein H aminomethyl carrier; Glycine cleavage system protein H; Lipoic acid containing protein; mitochondrial; Mitochondrial glycine cleavage system H protein; NKH.

    Background of the antigen

    GCSH is a 173 amino acid mitochondrial protein that contains one lipoyl-binding domain and belongs to the gcvH family. Defects in the gene encoding GCSH are the cause of glycine encephalopathy (GCE), an autosomal recessive disease that is also referred to as non-ketotic hyperglycinemia (NKH). Characterized by severe neurological symptoms, patients with GCE have a large amount of glycine accumulated in their body fluids. The gene encoding GCSH maps to human chromosome 16, which encodes over 900 genes and comprises nearly 3% of the human genome.

    Properties

    For facs or microscopy Alexa 1 conjugate.Alexa Fluor 488 has the same range to that of fluorescein isothiocyanate (FITC), yet the Anti-GCSH has a very high photo stability. As a result of this photo stability, it has turned into an antibody for fluorescent microscopy and FACS FLOW cytometry. It is distinguished in the FL1 of a FACS-Calibur or FACScan. Also Alexa Fluor 488 is pH stable.If you buy Antibodies supplied by Bioss Primary Conjugated Antibodies. ALEXA FLUOR they should be stored frozen at - 24°C for long term storage and for short term at + 5°C.