Anti-GDAP1 Antibody

$99.00$319.00

Reactivity: Human,Mouse
Applications: ELISA,WB,IHC
Conjugation: Various
Supplier: CUSABIO BIOTECH CO.

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Rabbit Anti-GDAP1 Antibody (CSB-PA009338ESR2HU)

Supplier: CUSABIO BIOTECH CO.

Alternative names:

Ganglioside-induced differentiation-associated protein 1 Antibody,GDAP1 Antibody,GDAP1 Antibody

More alternative names for the antibody
Charcot Marie Tooth neuropathy 4A antibody|CMT4 antibody|CMT4A antibody|CMTRIA antibody|Ganglioside induced differentiation associated protein 1 antibody|Ganglioside-induced differentiation-associated protein 1 antibody|GDAP1 antibody|GDAP1_HUMAN antibody
Anti-GDAP1 antibody (ab100905)
Close sc-86468|sc-98516|

Recommended applications: ELISA, WB, IHC

Recommended dilution: User optimized

Recommended protocols: check protocols

Name

Anti-GDAP1 Antibody

Catalogue No.

CSB-PA009338ESR2HU

Reactivity

Human, Mouse

Immunogen

Recombinant human Ganglioside-induced differentiation-associated protein 1 protein (1-225AA)

Host

Rabbit

Applications

ELISA, WB, IHC

Recommended dilution

User optimized

Clonality

Polyclonal

Conjugation

unconjugated

Isotype

IgG

Molecular weight

41 kDa

Purification

Antigen Affinity Purified

Storage

Shipped at 4 Celcius Degree. Upon delivery aliquot and store at -20 Celcius Degree or -80 Celcius Degree. Avoid repeated freeze.

Database links

Entrez Gene:613472( Cow), Entrez Gene:54332( Human), Entrez Gene:14545( Mouse), Entrez Gene:312890( Rat), Omim:606598( Human), SwissProt:A6QQZ0( Cow), SwissProt:Q8TB36( Human), SwissProt:O88741( Mouse), Unigene:168950( Human), Unigene:18218( Mouse)

Protein function

Regulates the mitochondrial network by promoting mitochondrial fission. .

Protein tissue specificity

Highly expressed in whole brain and spinal cord. Predominant expression in central tissues of the nervous system not only in neurons but also in Schwann cells. .

Involvement in disease

Charcot-Marie-Tooth disease 4A (CMT4A) [MIM:214400]: A recessive demyelinating form of Charcot-Marie-Tooth disease, a disorder of the peripheral nervous system, characterized by progressive weakness and atrophy, initially of the peroneal muscles and later of the distal muscles of the arms. Charcot-Marie-Tooth disease is classified in two main groups on the basis of electrophysiologic properties and histopathology: primary peripheral demyelinating neuropathies (designated CMT1 when they are dominantly inherited) and primary peripheral axonal neuropathies (CMT2). Demyelinating neuropathies are characterized by severely reduced nerve conduction velocities (less than 38 m/sec), segmental demyelination and remyelination with onion bulb formations on nerve biopsy, slowly progressive distal muscle atrophy and weakness, absent deep tendon reflexes, and hollow feet. By convention autosomal recessive forms of demyelinating Charcot-Marie-Tooth disease are designated CMT4. CMT4A is a severe form characterized by early age of onset and rapid progression leading to inability to walk in late childhood or adolescence. . Note=The disease is caused by mutations affecting the gene represented in this entry.; Charcot-Marie-Tooth disease, axonal, with vocal cord paresis, autosomal recessive (CMT2RV) [MIM:607706]: A form of Charcot-Marie-Tooth disease characterized by the association of axonal neuropathy with vocal cord paresis. Charcot-Marie-Tooth disease is a disorder of the peripheral nervous system, characterized by progressive weakness and atrophy, initially of the peroneal muscles and later of the distal muscles of the arms. . Note=The disease is caused by mutations affecting the gene represented in this entry.; Charcot-Marie-Tooth disease 2K (CMT2K) [MIM:607831]: An axonal form of Charcot-Marie-Tooth disease, a disorder of the peripheral nervous system, characterized by progressive weakness and atrophy, initially of the peroneal muscles and later of the distal muscles of the arms. Charcot-Marie-Tooth disease is classified in two main groups on the basis of electrophysiologic properties and histopathology: primary peripheral demyelinating neuropathies (designated CMT1 when they are dominantly inherited) and primary peripheral axonal neuropathies (CMT2). Neuropathies of the CMT2 group are characterized by signs of axonal degeneration in the absence of obvious myelin alterations, normal or slightly reduced nerve conduction velocities, and progressive distal muscle weakness and atrophy. Nerve conduction velocities are normal or slightly reduced. Charcot-Marie-Tooth disease type 2K onset is in early childhood (younger than 3 years). This phenotype is characterized by foot deformities, kyphoscoliosis, distal limb muscle weakness and atrophy, areflexia, and diminished sensation in the lower limbs. Weakness in the upper limbs is observed in the first decade, with clawing of the fingers. Inheritance can be autosomal dominant or recessive. . Note=The disease is caused by mutations affecting the gene represented in this entry.; Charcot-Marie-Tooth disease, recessive, intermediate type, A (CMTRIA) [MIM:608340]: A form of Charcot-Marie-Tooth disease, a disorder of the peripheral nervous system, characterized by progressive weakness and atrophy, initially of the peroneal muscles and later of the distal muscles of the arms. Recessive intermediate forms of Charcot-Marie-Tooth disease are characterized by clinical and pathologic features intermediate between demyelinating and axonal peripheral neuropathies, and motor median nerve conduction velocities ranging from 25 to 45 m/sec. . Note=The disease is caused by mutations affecting the gene represented in this entry.

Protein sequence and domain

Belongs to the GST superfamily.

Protein post-translational modifications

Ubiquitinated by PARK2 during mitophagy, leading to its degradation and enhancement of mitophagy. Deubiquitinated by USP30. .

Protein cellular localization

Mitochondrion outer membrane ; Multi-pass membrane protein . Cytoplasm .

Research area

All research areas>Synthesis and Degradation>GDAP
(View all antibody categories related to Synthesis and Degradation)

Note

AntibodyPlus provides antibody trial sample for your own antibody validation and collects antibody reviews.

Supplier

CUSABIO BIOTECH CO.

Product type

Primary antibody

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